PMID- 24633036 OWN - NLM STAT- In-Data-Review DA - 20140317 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 53 IP - 6 DP - 2014 TI - Successful use of intensive immunosuppressive therapy for treating simulta neously occurring cerebral lesions and pulmonary arterial hypertension in a patien t with systemic lupus erythematosus. PG - 627-31 AB - A 59-year-old woman who had been diagnosed with systemic lupus erythematos us (SLE) was admitted to our hospital due to paralysis in all of her limbs. T he patient presented with dysarthria, cerebellar ataxia and hypoxia. Magnetic resonance imaging (MRI) revealed vasogenic edema in the brain stem and the cerebellum. She was diagnosed with neuropsychiatric lupus syndrome (NPSLE) and pulmonary arterial hypertension (PAH), and was successfully treated using immunosuppressive therapy. To our knowledge, this is the first reported ca se of simultaneously developing NPSLE and PAH. FAU - Watanabe, Ryu AU - Watanabe R AD - Department of Hematology and Rheumatology, Tohoku University Graduate Scho ol of Medicine, Japan. FAU - Fujii, Hiroshi AU - Fujii H FAU - Shirai, Tsuyoshi AU - Shirai T FAU - Saito, Shinichiro AU - Saito S FAU - Hatakeyama, Akira AU - Hatakeyama A FAU - Sugimura, Koichiro AU - Sugimura K FAU - Fukumoto, Yoshihiro AU - Fukumoto Y FAU - Ishii, Tomonori AU - Ishii T FAU - Harigae, Hideo AU - Harigae H LA - eng PT - Journal Article PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 SB - IM EDAT- 2014/03/19 06:00 MHDA- 2014/03/19 06:00 CRDT- 2014/03/18 06:00 AID - DN/JST.JSTAGE/internalmedicine/53.0514 [pii] PST - ppublish

SO - Intern Med. 2014;53(6):627-31. PMIDOWN STATDA IS IS VI IP DP TI irin: 24520278 NLM Publisher 20140212 1792-0981 (Print) 1792-0981 (Linking) 7 3 2014 Mar Effectiveness of cilostazol in transient ischemic attack refractory to asp A report of two cases. PG - 739-741 AB - Transient ischemic attack (TIA) is a warning of impending ischemic stroke. It provides an important therapeutic time window in which appropriate interve ntion may prevent permanent neurological injury. The anti-platelet agent, aspiri n, is an option for reducing the risk of stroke following TIA. However, for pati ents who are not responsive to aspirin, cilostazol may be an effective treatmen t. The current study presents two cases of TIA that were refractory to aspirin bu t were successfully treated with cilostazol. In case 1, an 83-year-old female pat ient suffered from episodes of weakness and numbness of the left extremities. A spirin alone or aspirin in combination with clopidogrel were not effective. Anticoagulation therapy with low molecular heparin decreased the frequency of ischemic episodes with complete remission following antiplatelet therapy w ith cilostazol. In case 2, a 51-year-old male presentedwith episodes of paroxy smal weakness of the left extremities with dysarthria. Antiplatelet therapy wit h aspirin was initiated. Eight episodes of ischemic attack recurred on the s eventh day following admission. After the change of the antiplatelet agent to cilostazol, no ischemic episodes recurred, with the exception of three on the first day. This study suggested that cilostazol may be efficacious in the prevention of ischemic stoke following TIA of a non-cardiac origin that wa s not responsive to aspirin. FAU - Lin, Gaoping AU - Lin G AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou, Zhejiang 310014, P.R. China. FAU - Ren, Dongdong AU - Ren D AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou, Zhejiang 310014, P.R. China. FAU - Guo, Shunyuan AU - Guo S AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou,

Zhejiang 310014, P.R. China. FAU - Geng, Yu AU - Geng Y AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou, Zhejiang 310014, P.R. China. LA - ENG PT - JOURNAL ARTICLE DEP - 20131231 TA - Exp Ther Med JT - Experimental and therapeutic medicine JID - 101531947 PMC - PMC3919941 OTO - NOTNLM OT - aspirin OT - cilostazol OT - transient ischemic attack EDAT- 2014/02/13 06:00 MHDA- 2014/02/13 06:00 CRDT- 2014/02/13 06:00 PHST- 2013/08/07 [received] PHST- 2013/12/23 [accepted] PHST- 2013/12/31 [epublish] AID - 10.3892/etm.2013.1468 [doi] AID - etm-07-03-0739 [pii] PST - ppublish SO - Exp Ther Med. 2014 Mar;7(3):739-741. Epub 2013 Dec 31. PMIDOWN STATDA IS IS VI DP TI PG LID AB ions) 24438445 NLM In-Process 20140122 1471-2377 (Electronic) 1471-2377 (Linking) 14 2014 High-resolution MRI findings in patients with capsular warning syndrome. 16 10.1186/1471-2377-14-16 [doi] BACKGROUND: Capsular warning syndrome (CWS) is rare (1.5% of TIA presentat

but has a poor prognosis (7-day stroke risk of 60%). Up to date, the exact pathogenic mechanism of CWS has not been fully understood. We report the c linical presentations and high-resolution MRI (HR MRI) findings of two cases with capsular warning symptoms. CASE PRESENTATION: Case 1 was a 63-year-old man with a history of hypertension with recurrent episodes of left hemiparesis and dysarthria lasting 10 ~ 30 minutes. Case 2 was a 54-year-old woman with repetitive episodes of transient left hemiparesis and dysarthria lasting a bout 10 minutes. Capsular infarctions on DWI were demonstrated in the territory of a lenticulostriate artery in both 2 patients. HR MRI disclosed atherosclerot ic plaques on the ventral wall of the MCA where enticulostriate arteries were arisen from, although traditional digital subtraction angiography showed normal. Aggressive medical therapy with dual antithrombotic agents and statin was effective in these two cases. CONCLUSION: Our HR MRI data offer an insight into

the pathophysiology of CWS which might be caused by atherosclerotic plaque in non-stenotic MCA wall. HR MRI might be a useful modality for characterizin g atherosclerotic plaques in the MCA and detecting the pathophysiology of th e CWS. FAU - Zhou, Lixin AU - Zhou L FAU - Ni, Jun AU - Ni J FAU - Xu, Weihai AU - Xu W FAU - Yao, Ming AU - Yao M FAU - Peng, Bin AU - Peng B FAU - Li, Mingli AU - Li M FAU - Cui, Liying AU - Cui L AD - Department of Neurology, Peking Union Medical College Hospital and Chinese Academy of Medical Science, Shuai Fu Yuan 1#, Dong Cheng District, Beijing 100730, China. pumchcuily@sina.com. LA - eng PT - Journal Article DEP - 20140120 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 SB - IM PMC - PMC3898091 OID - NLM: PMC3898091 EDAT- 2014/01/21 06:00 MHDA- 2014/01/21 06:00 CRDT- 2014/01/21 06:00 PHST- 2013/09/04 [received] PHST- 2014/01/15 [accepted] PHST- 2014/01/20 [aheadofprint] AID - 1471-2377-14-16 [pii] AID - 10.1186/1471-2377-14-16 [doi] PST - epublish SO - BMC Neurol. 2014 Jan 20;14:16. doi: 10.1186/1471-2377-14-16. PMIDOWN STATDA IS IS DP TI e 24390182 NLM Publisher 20140106 1349-8029 (Electronic) 0470-8105 (Linking) 2013 Dec 27 Vertebrobasilar Infarction Related to Giant Cell (Temporal) Arteritis: Cas

Report. AB - An 84-year-old male with a 3-month history of headache and elevated C-reac tive protein levels was admitted for biopsy of the superficial temporal artery, which led to the diagnosis of giant cell arteritis (GCA). Two days after prednis olone

therapy was initiated, the patient began to experience transient vertigo a ttacks. Two days later, dysarthria, left-sided hemiparesis, right abducens palsy, and horizontal nystagmus developed. Magnetic resonance (MR) imaging disclosed fresh infarctions in the vertebrobasilar territory. Since the patient became dro wsy because of brainstem compression and hydrocephalus due to cerebellar swell ing, emergency suboccipital decompression surgery and ventricular drainage were performed. Subsequently, the patient's consciousness levels improved. MR angiography revealed right vertebral artery (VA) occlusion and left VA ste nosis due to arteritis. Ischemic stroke is a serious though relatively rare complication of GCA. Similar cases have been reported, in which ischemic s troke developed despite or possibly due to steroid therapy. To our knowledge, th is is the first description of vertebrobasilar infarction associated with GCA in the Japanese population. The merits and potential demerits of steroid therapy are briefly discussed. FAU - Haisa, Toshihiko AU - Haisa T AD - Department of Neurosurgery, JR Tokyo General Hospital. FAU - Tsuda, Tokutaro AU - Tsuda T FAU - Hagiwara, Kiyofumi AU - Hagiwara K FAU - Kikuchi, Takeshi AU - Kikuchi T FAU - Seki, Kunihiko AU - Seki K LA - ENG PT - JOURNAL ARTICLE DEP - 20131227 TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 EDAT- 2014/01/07 06:00 MHDA- 2014/01/07 06:00 CRDT- 2014/01/07 06:00 AID - DN/JST.JSTAGE/nmc/cr.2013-0038 [pii] PST - aheadofprint SO - Neurol Med Chir (Tokyo). 2013 Dec 27. PMIDOWN STATDA IS IS VI DP TI PG LID AB 24359465 NLM In-Process 20140106 1471-2377 (Electronic) 1471-2377 (Linking) 13 2013 Marchiafava-Bignami disease mimics motor neuron disease: case report. 208 10.1186/1471-2377-13-208 [doi] BACKGROUND: Marchiafava-Bignami disease (MBD) is a rare neurologic complic

ation of chronic alcohol consumption that is characterized by callosal lesions involving demyelination and necrosis. Various reversible neurologic sympto ms are found in patients with MBD. Dysarthria and dysphagia are found in various neurological diseases. CASE PRESENTATION: We report a 51-year-old man with chronic alcoholism and malnutrition who progressively developed dysarthria and dysphagia. On admission, the patient was alert with mild cognitive dysfunc tion. The facial expression was flat, and there was weakness of the orbicularis oris bilaterally. The patient's speech was slurred, there was difficulty swallo wing, and the gag reflex and palate elevation were poor. The jaw jerk reflex was brisk and the snout reflex was positive. Neither tongue atrophy nor fasciculatio n were found. Bilateral upper and lower limb weakness with increased bilateral up per limb reflexes and Babinski reflexes were found. Because he had progressive dysarthria and dysphagia with upper and lower motor neuron signs, the init ial diagnosis was motor neuron disease. However, electrophysiological analysis was normal. The vitamin B1 level was 14 ng/mL (normal: >24 ng/mL), and MRI rev ealed hyperintense lesions in the splenium of the corpus callosum and the primar y motor cortices bilaterally. After vitamin B therapy for 17 days, the neurologica l disorders alleviated concurrently with disappearance of the lesions on MRI , which led to the definitive diagnosis of MBD. CONCLUSIONS: MBD presenting with t hese lesions can mimic motor neuron disease clinically. FAU - Hoshino, Yasunobu AU - Hoshino Y FAU - Ueno, Yuji AU - Ueno Y AD - Department of Neurology, Juntendo University Urayasu Hospital, 2-1-1 Tomio ka, Urayasu, Chiba 279-0021, Japan. yuji-u@juntendo.ac.jp. FAU - Shimura, Hideki AU - Shimura H FAU - Miyamoto, Nobukazu AU - Miyamoto N FAU - Watanabe, Masao AU - Watanabe M FAU - Hattori, Nobutaka AU - Hattori N FAU - Urabe, Takao AU - Urabe T LA - eng PT - Journal Article DEP - 20131221 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555

SB PMC OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO -

IM PMC3880166 NLM: PMC3880166 2013/12/24 06:00 2013/12/24 06:00 2013/12/24 06:00 2013/09/19 [received] 2013/12/17 [accepted] 2013/12/21 [aheadofprint] 1471-2377-13-208 [pii] 10.1186/1471-2377-13-208 [doi] epublish BMC Neurol. 2013 Dec 21;13:208. doi: 10.1186/1471-2377-13-208.

PMID- 24316510 OWN - NLM STAT- MEDLINE DA - 20140115 DCOM- 20140311 IS - 1460-2156 (Electronic) IS - 0006-8950 (Linking) VI - 137 IP - Pt 1 DP - 2014 Jan TI - Pantethine treatment is effective in recovering the disease phenotype indu ced by ketogenic diet in a pantothenate kinase-associated neurodegeneration mouse model. PG - 57-68 LID - 10.1093/brain/awt325 [doi] AB - Pantothenate kinase-associated neurodegeneration, caused by mutations in t he PANK2 gene, is an autosomal recessive disorder characterized by dystonia, dysarthria, rigidity, pigmentary retinal degeneration and brain iron accumulation. PANK2 encodes the mitochondrial enzyme pantothenate kinase t ype 2, responsible for the phosphorylation of pantothenate or vitamin B5 in the biosynthesis of co-enzyme A. A Pank2 knockout (Pank2(-/-)) mouse model did not recapitulate the human disease but showed azoospermia and mitochondrial dysfunctions. We challenged this mouse model with a low glucose and high l ipid content diet (ketogenic diet) to stimulate lipid use by mitochondrial beta-oxidation. In the presence of a shortage of co-enzyme A, this diet co uld evoke a general impairment of bioenergetic metabolism. Only Pank2(-/-) mic e fed with a ketogenic diet developed a pantothenate kinase-associated neurodegeneration-like syndrome characterized by severe motor dysfunction, neurodegeneration and severely altered mitochondria in the central and per ipheral nervous systems. These mice also showed structural alteration of muscle morphology, which was comparable with that observed in a patient with pantothenate kinase-associated neurodegeneration. We here demonstrate that pantethine administration can prevent the onset of the neuromuscular pheno type in mice suggesting the possibility of experimental treatment in patients with pantothenate kinase-associated neurodegeneration. FAU - Brunetti, Dario AU - Brunetti D

AD - 1 Unit of Molecular Neurogenetics, Foundation IRCCS Neurological Institute C. Besta, Milan, Italy. FAU - Dusi, Sabrina AU - Dusi S FAU - Giordano, Carla AU - Giordano C FAU - Lamperti, Costanza AU - Lamperti C FAU - Morbin, Michela AU - Morbin M FAU - Fugnanesi, Valeria AU - Fugnanesi V FAU - Marchet, Silvia AU - Marchet S FAU - Fagiolari, Gigliola AU - Fagiolari G FAU - Sibon, Ody AU - Sibon O FAU - Moggio, Maurizio AU - Moggio M FAU - d'Amati, Giulia AU - d'Amati G FAU - Tiranti, Valeria AU - Tiranti V LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131206 PL - England TA - Brain JT - Brain : a journal of neurology JID - 0372537 RN - 0 (Triglycerides) RN - 496-65-1 (Pantetheine) RN - 7K81IL792L (pantethine) RN - 97C5T2UQ7J (Cholesterol) RN - EC 2.7.1.- (Phosphotransferases (Alcohol Group Acceptor)) RN - EC 2.7.1.33 (pantothenate kinase) SB - AIM SB - IM CIN - Brain. 2014 Jan;137(Pt 1):8-11. PMID: 24424915 MH - Animals MH - Behavior, Animal/physiology MH - Brain/pathology MH - Cholesterol/blood MH - Energy Metabolism/physiology MH - Female MH - Heredodegenerative Disorders, Nervous System/*genetics/physiopathology/psy chology MH - Immunohistochemistry MH - Ketogenic Diet/*adverse effects MH - Male MH - Membrane Potential, Mitochondrial/physiology MH - Mice MH - Mice, Knockout MH - Microscopy, Electron MH - Mitochondria/pathology MH - Motor Skills/physiology MH - Neurons/pathology

MH MH MH MH MH MH PMC OID OTO OT OT OT OT EDATMHDACRDTPHSTAID AID PST SO 6. PMIDOWN STATDA IS IS VI IP DP TI -

Pantetheine/*analogs & derivatives/therapeutic use Peripheral Nervous System/pathology/physiopathology Phenotype Phosphotransferases (Alcohol Group Acceptor)/*genetics/physiology Sciatic Nerve/pathology Triglycerides/blood PMC3891449 NLM: PMC3891449 NOTNLM ketogenic diet mitochondria pantethine pantothenate kinase-associated neurodegeneration (PKAN) 2013/12/10 06:00 2014/03/13 06:00 2013/12/10 06:00 2013/12/06 [aheadofprint] awt325 [pii] 10.1093/brain/awt325 [doi] ppublish Brain. 2014 Jan;137(Pt 1):57-68. doi: 10.1093/brain/awt325. Epub 2013 Dec

24223913 NLM In-Process 20131113 1932-6203 (Electronic) 1932-6203 (Linking) 8 11 2013 Short and long term outcome of bilateral pallidal stimulation in chorea-acanthocytosis. PG - e79241 LID - 10.1371/journal.pone.0079241 [doi] AB - BACKGROUND: Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therap y. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these pa tients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS . METHODS: Data were collected in a standardized way 2-6 months preoperative ly, 1-5 months (early) and 6 months or more (late) after surgery at the last follo w-up visit (mean follow-up: 29.5 months). RESULTS: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mea n improvement 54.3% and 44.1%, respectively). Functional capacity (UHDRS-Fun ctional Capacity Score) was also significantly improved at both post-surgery time points (mean 75.5% and 73.3%, respectively), whereas incapacity (UHDRS-Independen ce

Score) improvement reached significance at early post-surgery only (mean 3 7.3%). Long term significant improvement of motor symptom severity (>/= 20 % from baseline) was observed in 61.5 % of the patients. Chorea and dystonia impr oved, whereas effects on dysarthria and swallowing were variable. Parkinsonism d id not improve. Linear regression analysis showed that preoperative motor severit y predicted motor improvement at both post-surgery time points. The most ser ious adverse event was device infection and cerebral abscess, and one patient d ied suddenly of unclear cause, 4 years after surgery. CONCLUSION: This study s hows that bilateral DBS of the GPi effectively reduces the severity of drug-res istant hyperkinetic movement disorders such as present in ChAc. FAU - Miquel, Marie AU - Miquel M AD - Service de Neurologie, CHU Bordeaux, Bordeaux, France ; Service de Neurolo gie, CH Francois Mitterrand, Pau, France. FAU - Spampinato, Umberto AU - Spampinato U FAU - Latxague, Chrystelle AU - Latxague C FAU - Aviles-Olmos, Iciar AU - Aviles-Olmos I FAU - Bader, Benedikt AU - Bader B FAU - Bertram, Kelly AU - Bertram K FAU - Bhatia, Kailash AU - Bhatia K FAU - Burbaud, Pierre AU - Burbaud P FAU - Burghaus, Lothar AU - Burghaus L FAU - Cho, Jin Whan AU - Cho JW FAU - Cuny, Emmanuel AU - Cuny E FAU - Danek, Adrian AU - Danek A FAU - Foltynie, Thomas AU - Foltynie T FAU - Garcia Ruiz, Pedro J AU - Garcia Ruiz PJ FAU - Gimenez-Roldan, Santiago AU - Gimenez-Roldan S FAU - Guehl, Dominique AU - Guehl D FAU - Guridi, Jorge AU - Guridi J FAU - Hariz, Marwan AU - Hariz M FAU - Jarman, Paul AU - Jarman P FAU - Kefalopoulou, Zinovia Maria

AU - Kefalopoulou ZM FAU - Limousin, Patricia AU - Limousin P FAU - Lipsman, Nir AU - Lipsman N FAU - Lozano, Andres M AU - Lozano AM FAU - Moro, Elena AU - Moro E FAU - Ngy, Dhita AU - Ngy D FAU - Rodriguez-Oroz, Maria Cruz AU - Rodriguez-Oroz MC FAU - Shang, Huifang AU - Shang H FAU - Shin, Hyeeun AU - Shin H FAU - Walker, Ruth H AU - Walker RH FAU - Yokochi, Fusako AU - Yokochi F FAU - Zrinzo, Ludvic AU - Zrinzo L FAU - Tison, Francois AU - Tison F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131105 PL - United States TA - PLoS One JT - PloS one JID - 101285081 SB - IM PMC - PMC3818425 OID - NLM: PMC3818425 EDAT- 2013/11/14 06:00 MHDA- 2013/11/14 06:00 CRDT- 2013/11/14 06:00 PHST- 2013 [ecollection] PHST- 2013/07/15 [received] PHST- 2013/09/19 [accepted] PHST- 2013/11/05 [epublish] AID - 10.1371/journal.pone.0079241 [doi] AID - PONE-D-13-29059 [pii] PST - epublish SO - PLoS One. 2013 Nov 5;8(11):e79241. doi: 10.1371/journal.pone.0079241. eCol lection 2013. PMID- 24135395 OWN - NLM STAT- In-Process DA - 20140116 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 8 DP - 2013 TI - Niemann-Pick disease type C symptomatology: an expert-based clinical descr iption.

PG - 166 LID - 10.1186/1750-1172-8-166 [doi] AB - Niemann-Pick disease type C (NP-C) is a rare, progressive, irreversible di sease leading to disabling neurological manifestations and premature death. The estimated disease incidence is 1:120,000 live births, but this likely repr esents an underestimate, as the disease may be under-diagnosed due to its highly heterogeneous presentation. NP-C is characterised by visceral, neurologica l and psychiatric manifestations that are not specific to the disease and that c an be found in other conditions. The aim of this review is to provide non-specia lists with an expert-based, detailed description of NP-C signs and symptoms, inc luding how they present in patients and how they can be assessed. Early disease detection should rely on seeking a combination of signs and symptoms, rath er than isolated findings. Examples of combinations which are strongly suggestive of NP-C include: splenomegaly and vertical supranuclear gaze palsy (VSGP); splenom egaly and clumsiness; splenomegaly and schizophrenia-like psychosis; psychotic s ymptoms and cognitive decline; and ataxia with dystonia, dysarthria/dysphagia and cognitive decline. VSGP is a hallmark of NP-C and becomes highly specific of the disease when it occurs in combination with other manifestations (e.g. splenomegaly, ataxia). In young infants (6 years o f age). Psychosis in NP-C is atypical and variably responsive to treatment. Progre ssive cognitive decline, which always occurs in patients with NP-C, manifests as memory and executive impairment in juvenile/adult patients. Disease prognosis mai nly correlates with the age at onset of the neurological signs, with early-ons et forms progressing faster. Therefore, a detailed and descriptive picture of NP-C signs and symptoms may help improve disease detection and early diagnosis, so that therapy with miglustat (Zavesca((R))), the only available treatment a pproved to date, can be started as soon as neurological symptoms appear, in order to slow disease progression. FAU - Mengel, Eugen AU - Mengel E AD - Department of Lysosomal Storage Disorder, Villa Metabolica, Center for Pae diatric and Adolescent Medicine, University Medical Center of the Johannes Gutenbe

rg FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU LA PT PT DEP PL TA JT JID SB PMC OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA DCOMIS IS VI DP TI ome PG LID AB als oman carrying a homozygous mutation (p.A899T) in mitochondrial polymerase gamma (POLG) and manifesting with a complex neurological phenotype including Dopamine-a gonist University Mainz, Langenbeckstrasse 1, 55131 Mainz, Germany. mengel@kinder.klinik.uni-mainz.de. Klunemann, Hans-Hermann Klunemann HH Lourenco, Charles M Lourenco CM Hendriksz, Christian J Hendriksz CJ Sedel, Frederic Sedel F Walterfang, Mark Walterfang M Kolb, Stefan A Kolb SA eng Journal Article Research Support, Non-U.S. Gov't 20131017 England Orphanet J Rare Dis Orphanet journal of rare diseases 101266602 IM PMC3853996 NLM: PMC3853996 2013/10/19 06:00 2013/10/19 06:00 2013/10/19 06:00 2013/06/04 [received] 2013/10/01 [accepted] 2013/10/17 [aheadofprint] 1750-1172-8-166 [pii] 10.1186/1750-1172-8-166 [doi] epublish Orphanet J Rare Dis. 2013 Oct 17;8:166. doi: 10.1186/1750-1172-8-166. 24099403 NLM MEDLINE 20131115 20140106 1471-2350 (Electronic) 1471-2350 (Linking) 14 2013 Dopamine-agonist responsive Parkinsonism in a patient with the SANDO syndr

caused by POLG mutation. - 105 - 10.1186/1471-2350-14-105 [doi] - BACKGROUND: Disorders of oxidative phosphorylation affects 1/5000 individu and present heterogeneous involvement of tissues highly dependent upon ATP production. CASE PRESENTATION: Here we present the case of a 48-year-old w

responsive Parkinsonism. CONCLUSION: This case report is further evidence that mitochondrial dysfunction might play a role in Parkinson's Disease pathoge nesis and helps in identification of apparent mutation-specific clinical characteristics. Mutations in POLG should be looked for in cases of Parkin sonism, especially when multisystem neurological involvement is found. FAU - Bandettini di Poggio, Monica AU - Bandettini di Poggio M AD - Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Mater nal and Child Health, University of Genova and IRCSS Azienda Opedaliera Universita ria San Martino-IST, Largo Daneo 3-16132, Genova, Italy. monicabandettini@yahoo.it . FAU - Nesti, Claudia AU - Nesti C FAU - Bruno, Claudio AU - Bruno C FAU - Meschini, Maria Chiara AU - Meschini MC FAU - Schenone, Angelo AU - Schenone A FAU - Santorelli, Filippo M AU - Santorelli FM LA - eng PT - Case Reports PT - Journal Article DEP - 20131007 PL - England TA - BMC Med Genet JT - BMC medical genetics JID - 100968552 RN - 0 (Benzothiazoles) RN - 0 (Dopamine Agonists) RN - 0 (Thiophenes) RN - 83619PEU5T (pramipexole) RN - EC 2.7.7.- (POLG protein, human) RN - EC 2.7.7.7 (DNA-Directed DNA Polymerase) RN - O5TNM5N07U (duloxetine) RN - Sensory ataxic neuropathy, dysarthria, and ophthalmoparesis SB - IM MH - Benzothiazoles/therapeutic use MH - DNA Mutational Analysis MH - DNA-Directed DNA Polymerase/*genetics/metabolism MH - Dopamine Agonists/*therapeutic use MH - Dysarthria/complications/*genetics/pathology MH - Female MH - Hereditary Sensory and Motor Neuropathy/complications/*genetics/pathology MH - Humans MH - Middle Aged MH - Mitochondria/enzymology MH - Ophthalmoplegia/complications/*genetics/pathology MH - Parkinson Disease/complications/*drug therapy MH - Thiophenes/therapeutic use MH - Tomography, Emission-Computed, Single-Photon PMC - PMC3851930 OID - NLM: PMC3851930 EDAT- 2013/10/09 06:00

MHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA IS IS VI IP DP TI ance

2014/01/07 06:00 2013/10/09 06:00 2013/03/05 [received] 2013/09/25 [accepted] 2013/10/07 [aheadofprint] 1471-2350-14-105 [pii] 10.1186/1471-2350-14-105 [doi] epublish BMC Med Genet. 2013 Oct 7;14:105. doi: 10.1186/1471-2350-14-105. 24081890 NLM In-Process 20131001 1576-6578 (Electronic) 0210-0010 (Linking) 57 8 2013 Oct 16 [CLIPPERS syndrome with atypical distribution of lesions in magnetic reson

imaging of the brain]. PG - 354-8 AB - INTRODUCTION: CLIPPERS syndrome (chronic lymphocytic inflammation with pon tine perivascular enhancement responsive to steroids) is an inflammatory proces s of the central nervous system whose distinguishing features are the enhancing punctiform lesions in the brainstem that appear in the magnetic resonance images. Clinically, it is accompanied by dysarthria, ataxia and diplopia, and usua lly responds to treatment with corticoids. Pathologically, T lymphocytes appea r infiltrated in the perivascular spaces of the brainstem. CASE REPORT: We r eport the case of a 40-year-old woman with an initial subacute clinical picture of binocular diplopia, ataxia and dysarthria. The magnetic resonance brain sc an revealed T2 hyperintense punctiform lesions in the stem, cerebellum, diencephalons and cortico-subcortical areas of both hemispheres, which wer e enhanced with contrast. An aetiological study was performed to rule out an y underlying infectious, neoplastic or inflammatory origin, the results bein g negative. The patient was treated on two occasions with methylprednisolone , with a gradual lowering of the dosage, the response being favourable. CONCLUSIO NS: Diplopia and ataxia, as in our case, are practically always present. The M R findings consist of punctiform enhancing lesions located in the pons exten ding towards the cerebellum, basal ganglia and corpus callosum, the enhancement gradient becoming lower as the distance increases rostrally away from the cortex, and caudally towards the spinal cord. In the case of our patient, this gra dient

is not respected, and the density found was similar to that of lesions at the supratentorial level. The differential diagnosis is wide-ranging and justi fies an extensive diagnostic study with, in certain cases, a biopsy study of brain tissue. The disease courses in a relapsing-remitting pattern and the earli er steroid therapy is established and the more prolonged it is, the better th e prognosis will be. FAU - Canneti, Beatrice AU - Canneti B AD - Hospital Universitario de la Princesa, 28006 Madrid, Espana. FAU - Mosqueira, Antonio J AU - Mosqueira AJ FAU - Gilo, Francisco AU - Gilo F FAU - Carreras, Teresa AU - Carreras T FAU - Barbosa, Antonio AU - Barbosa A FAU - Meca-Lallana, Virginia AU - Meca-Lallana V FAU - Vivancos, Jose AU - Vivancos J LA - spa PT - English Abstract PT - Journal Article TT - Sindrome CLIPPERS con distribucion atipica de las lesiones en la resonanci a magnetica cerebral. PL - Spain TA - Rev Neurol JT - Revista de neurologia JID - 7706841 SB - IM OAB - Publisher: Abstract available from the publisher. EDAT- 2013/10/02 06:00 MHDA- 2013/10/02 06:00 CRDT- 2013/10/02 06:00 AID - rn2013126 [pii] PST - ppublish SO - Rev Neurol. 2013 Oct 16;57(8):354-8. PMIDOWN STATDA IS IS VI DP TI 24067156 NLM In-Process 20131115 1471-2377 (Electronic) 1471-2377 (Linking) 13 2013 Unusual epileptic deterioration and extensive white matter lesion during treatment in Wilson's disease. PG - 127 LID - 10.1186/1471-2377-13-127 [doi] AB - BACKGROUND: Wilson's disease (WD) is a genetic disorder which can be contr olled fairly well with decupuration therapy. However, symptoms, on rare occasion s, can

worsen even when WD is being treated. Herein, we report a case involving u nusual neurological deterioration during decupuration therapy for WD. CASE PRESEN TATION: A 28-year-old man was diagnosed with WD 13 years prior to his clinical vis it; however, his drug compliance has been poor over the years. He was treated with trientine because tremors and dysarthria have presented in recent years. H owever, dysarthria and dystonia developed in his limbs, which were worse on the ri ght side and had been aggravated for several weeks despite good drug complianc e. His symptoms were fluctuating. It was initially misdiagnosed as dystonia; alth ough, it turned out to be a seizure due to cortical degeneration. These symptoms were completely resolved with antiepileptic drugs. Moreover, the cortical enhan cement of bifrontal degeneration has disappeared on the MRI. CONCLUSION: This cas e showed unusual epileptic neurologic deterioration due to cortical degenera tion during decupuration therapy. Seizures in WD can easily be mistaken as part of dystonia. However, the fluctuating symptoms suggest a seizure. FAU - Kim, Young Eun AU - Kim YE AD - Department of Neurology, Ulsan University Hospital, Ulsan, Korea. brain@snu.ac.kr. FAU - Yun, Ji Young AU - Yun JY FAU - Yang, Hui-Jun AU - Yang HJ FAU - Kim, Han-Joon AU - Kim HJ FAU - Jeon, Beom S AU - Jeon BS LA - eng PT - Journal Article DEP - 20130925 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 SB - IM PMC - PMC3851542 OID - NLM: PMC3851542 EDAT- 2013/09/27 06:00 MHDA- 2013/09/27 06:00 CRDT- 2013/09/27 06:00 PHST- 2012/09/21 [received] PHST- 2013/09/03 [accepted] PHST- 2013/09/25 [aheadofprint] AID - 1471-2377-13-127 [pii] AID - 10.1186/1471-2377-13-127 [doi] PST - epublish SO - BMC Neurol. 2013 Sep 25;13:127. doi: 10.1186/1471-2377-13-127.

PMIDOWN STATDA DCOMIS IS VI IP DP TI ing

23812076 NLM MEDLINE 20130701 20140228 1349-7413 (Electronic) 0911-4300 (Linking) 36 3 2013 [Diagnostic value of brain biopsy in a pediatric multiple sclerosis mimick

brain stem glioma]. PG - 175-9 AB - Diagnosis of multiple sclerosis (MS) is difficult when the lesion mimics g lioma or cerebral enchephalitis. We report a case of pediatric MS initially susp ected as brain stem glioma. An 11-year-old boy developed left foot joint pain fo llowed by progressive symptoms such as left arm and leg weakness, dysarthria, paraplegia, and decreased level of consciousness. He subsequently develope d respiratory distress requiring endotracheal intubation and mechanical ventilation. Magnetic resonance imaging showed a mass measuring 2 cm in th e medulla oblongata. Although this mass was initially suspected as a glioma, the patient's acutely progressive disease course was not consistent with this diagnosis. Open biopsy revealed inflammation and demyelination, but no mal ignant cells were detected. He was treated with steroid pulse therapy, which show ed dramatic effects. Nine months later, he developed another episode characte rized by several neurological symptoms, and the diagnosis of MS was clinically confirmed. Open brain stem biopsy is technically demanding, but this case demonstrates that appropriate neurosurgical evaluation can play an importa nt role in diagnosis by ruling out glioma and confirming MS. FAU - Nakazawa, Yumiko AU - Nakazawa Y AD - Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development. FAU - Maekawa, Takanobu AU - Maekawa T FAU - Oana, Shinji AU - Oana S FAU - Ishiguro, Akira AU - Ishiguro A FAU - Ohta, Sayaka AU - Ohta S FAU - Terashima, Hiroshi AU - Terashima H FAU - Kashii, Hirofumi AU - Kashii H FAU - Kubota, Masaya AU - Kubota M FAU - Tsutsumi, Yoshiyuki

AU FAU AU FAU AU FAU AU LA PT PT PT PL TA JT gy JID SB MH MH MH MH MH MH MH MH MH EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMIS IS VI DP TI f the

Tsutsumi Y Nakazawa, Atsuko Nakazawa A Morota, Nobuhito Morota N Sakai, Hirokazu Sakai H jpn Case Reports English Abstract Journal Article Japan Nihon Rinsho Meneki Gakkai Kaishi Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunolo 9505992 IM Biopsy Brain/*pathology Brain Stem Neoplasms/*pathology Child *Diagnosis, Differential Glioma/*pathology Humans Male Multiple Sclerosis/*pathology 2013/07/03 06:00 2014/03/01 06:00 2013/07/02 06:00 DN/JST.JSTAGE/jsci/36.175 [pii] ppublish Nihon Rinsho Meneki Gakkai Kaishi. 2013;36(3):175-9. 23742248 NLM MEDLINE 20130626 20140102 1750-1172 (Electronic) 1750-1172 (Linking) 8 2013 Biotin-responsive basal ganglia disease should be renamed biotin-thiamine-responsive basal ganglia disease: a retrospective review o

clinical, radiological and molecular findings of 18 new cases. PG - 83 LID - 10.1186/1750-1172-8-83 [doi] AB - BACKGROUND: Biotin-responsive basal ganglia disease (BBGD) is an autosomal recessive neurometabolic disorder. It is characterized by sub acute encephalopathy with confusion, seizure, dysarthria and dystonia following a history of febrile illness. If left untreated with biotin, the disease can progress to severe quadriparesis and even death. METHOD: A retrospective c hart review of 18 patients with BBGD from two tertiary institutions describing their clinical, magnetic resonance imaging and molecular findings was conducted. RESULT: Eighteen children from 13 families seen over a period of nine year s

(2003-2012) were included. (Age range: 14month to 23 years, M: F: 1:1). Th e clinical features included sub acute encephalopathy, ataxia (n= 18), seizu res (n= 13) dystonia (n=12) ,dysarthria (n= 9), quadriparesis and hyperreflexia (n =9). Magnetic resonance imaging demonstrated abnormal signal intensity with swe lling in the basal ganglia during acute crises (n= 13/13) and atrophy of the bas al ganglia and necrosis during follow up (n= 13/13). One-third of the present patients showed the recurrence of acute crises while on biotin therapy alo ne, but after the addition of thiamine, crises did not recur. All of the patients have a homozygous missense mutation in exon 5 of the SLC19A3 gene. The frequency of acute crises, delay in diagnosis and initiation of treatment significantly influenced the outcome. On follow up, four patients died, two had spastic quadriplegia, six had normal outcome and the rest had speech and motor dysfunctions. CONCLUSION: Clinicians should suspect BBGD in any child pres enting with sub acute encephalopathy, abnormal movement and MRI findings as descr ibed above. Both biotin and thiamine are essential for disease management. Sinc e biotin alone could not prevent the recurrence of crises in some patients, a more appropriate term to describe the disease would be biotin-thiamine-responsi ve basal ganglia disease (BTBGD). FAU - Alfadhel, Majid AU - Alfadhel M AD - Division of Genetics, Department of Pediatrics, King Abdulaziz Medical Cit y, Riyadh, Saudi Arabia. dralfadhel@yahoo.com FAU - Almuntashri, Makki AU - Almuntashri M FAU - Jadah, Raafat H AU - Jadah RH FAU - Bashiri, Fahad A AU - Bashiri FA FAU - Al Rifai, Muhammad Talal AU - Al Rifai MT FAU - Al Shalaan, Hisham AU - Al Shalaan H FAU - Al Balwi, Mohammed AU - Al Balwi M FAU - Al Rumayan, Ahmed AU - Al Rumayan A FAU - Eyaid, Wafaa AU - Eyaid W FAU - Al-Twaijri, Waleed AU - Al-Twaijri W LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20130606 PL - England

TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (Membrane Transport Proteins) RN - 0 (SLC19A3 protein, human) RN - 6SO6U10H04 (Biotin) RN - X66NSO3N35 (Thiamine) RN - Basal ganglia disease, biotin-responsive SB - IM MH - Adolescent MH - Adult MH - Basal Ganglia/pathology MH - Basal Ganglia Diseases/*classification/*drug therapy/physiopathology/radio graphy MH - Biotin/therapeutic use MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Infant MH - Magnetic Resonance Imaging MH - Male MH - Membrane Transport Proteins/genetics MH - Mutation MH - Thiamine/*therapeutic use MH - Wernicke Encephalopathy/drug therapy/physiopathology/radiography MH - Young Adult PMC - PMC3691666 OID - NLM: PMC3691666 EDAT- 2013/06/08 06:00 MHDA- 2014/01/03 06:00 CRDT- 2013/06/08 06:00 PHST- 2013/02/14 [received] PHST- 2013/05/21 [accepted] PHST- 2013/06/06 [aheadofprint] AID - 1750-1172-8-83 [pii] AID - 10.1186/1750-1172-8-83 [doi] PST - epublish SO - Orphanet J Rare Dis. 2013 Jun 6;8:83. doi: 10.1186/1750-1172-8-83. PMIDOWN STATDA DCOMLR IS VI DP TI e 23734128 NLM PubMed-not-MEDLINE 20130604 20130605 20130813 1663-9812 (Electronic) 4 2013 Burden of Friedreich's Ataxia to the Patients and Healthcare Systems in th

United States and Canada. PG - 66 LID - 10.3389/fphar.2013.00066 [doi] AB - Objective: The study intended to substantiate healthcare resource utilizat ion, costs, and funding patterns of US and Canadian Friedreich's Ataxia (FRDA) populations, to assess compliance with treatment guidance and to identify areas where novel healthcare measures or improved access to existing care may im

prove patients' functional and social capabilities and reduce the financial impa ct on the healthcare systems. Methods: Healthcare resource utilization and costs were collected in a cross-sectional study in the US (N = 197) and Canada (N = 4 3) and analyzed across severity of disease categories. Descriptive statistics, correlation analysis, and hypothesis testing were applied. Results: In the US, healthcare costs of FRDA patients were higher than those of "adults with t wo and more chronic conditions." Significantly higher costs were incurred in adva nced stages of the disease, with paid homecare being the main driver. This patt ern was also observed in Canada. Compliance with the recommended annual neurologic al and cardiological follow-up was high, but was low for the recommended regular speech therapy. In the US public and private funding ratios were similar for the FRDA and the general populations. In Canada the private funding ratio for FRDA was higher than average. Conclusion: The variety of healthcare measures addres sing the broad range of symptoms of FRDA, and the increasing use of paid home c are as disease progresses made total US healthcare costs of FRDA exceed the costs of US adults with two and more chronic conditions. Therefore, measures delaying disease progression will allow patients to maintain their independence longer and may reduce costs to the healthcare system. Novel measures to address dysarthri a and to ensure access to them should be further investigated. The higher than a verage private funding ratio in Canada was due to the relatively high cost of the pharmacological treatment of FRDA. FAU - Polek, Barbara AU - Polek B AD - Santhera Pharmaceuticals Ltd Liestal, Switzerland. FAU - Roach, M J AU - Roach MJ FAU - Andrews, William T AU - Andrews WT FAU - Ehling, Manfred AU - Ehling M FAU - Salek, Sam AU - Salek S LA - eng PT - Journal Article DEP - 20130522 PL - Switzerland TA - Front Pharmacol JT - Frontiers in pharmacology JID - 101548923 PMC - PMC3660667 OID - NLM: PMC3660667

OTO OT OT OT OT OT OT EDATMHDACRDTPHSTPHSTPHSTPHSTAID PST SO ion PMIDOWN STATDA DCOMLR IS IS VI IP DP TI (110

NOTNLM Friedreich ataxia cost of illness cross-sectional studies healthcare resource rare diseases resource utilization 2013/06/05 06:00 2013/06/05 06:01 2013/06/05 06:00 2013 [ppublish] 2013/03/06 [received] 2013/04/29 [accepted] 2013/05/22 [epublish] 10.3389/fphar.2013.00066 [doi] epublish Front Pharmacol. 2013 May 22;4:66. doi: 10.3389/fphar.2013.00066. eCollect 2013. 23687505 NLM PubMed-not-MEDLINE 20130520 20130521 20130522 1664-5456 (Electronic) 1664-5456 (Linking) 3 1 2013 Jan Increase in the Size of an Intracardiac Thrombus during Dabigatran Therapy

mg b.i.d.) in an Acute Cardioembolic Stroke Patient. PG - 78-80 LID - 10.1159/000351137 [doi] AB - We report a case of atrial fibrillation in a patient in whom a mobile thro mbus in the left atrial appendage increased in size after low-dose dabigatran ther apy. A 74-year-old man was admitted to our hospital because of sudden onset of ri ght hemiplasia and dysarthria. On admission, his National Institutes of Health Stroke Scale score was three. Axial diffusion-weighted magnetic resonance images and magnetic resonance angiography images showed hyperintense signals in the l eft front-parietal cerebral cortex without any intracranial stenotic lesions, and acute cardioembolic stroke associated with nonvalvular atrial fibrillation was diagnosed. Transesophageal echocardiography revealed a mobile thrombosis ( 1.0 x 2.2 cm) in the left atrial appendage, and dabigatran therapy (110 mg b.i.d .) was initiated to prevent stroke recurrence. Transesophageal echocardiography performed 6 days later revealed that the size of the thrombus had increase d to 1.5 x 3.0 cm. Medication was changed to warfarin, and the thrombosis subse

quently decreased in size. The patient did not have a recurrent stroke and was dis charged with a National Institutes of Health Stroke Scale score of zero. This case demonstrates that low-dose dabigatran may not be effective in reducing the size of a thrombus. FAU - Tabata, Emi AU - Tabata E AD - Department of Cerebrovascular Medicine and Clinical Research Institute, Na tional Hospital Organization Kyushu Medical Center, Fukukuoka, Japan. FAU - Yasaka, Masahiro AU - Yasaka M FAU - Wakugawa, Yoshiyuki AU - Wakugawa Y FAU - Komori, Motohiro AU - Komori M FAU - Mori, Kohta AU - Mori K FAU - Tsurusaki, Yuichiro AU - Tsurusaki Y FAU - Kokuba, Kazuhito AU - Kokuba K FAU - Sambongi, Yoshiki AU - Sambongi Y FAU - Maeda, Koichiro AU - Maeda K FAU - Okada, Yasushi AU - Okada Y LA - eng PT - Journal Article DEP - 20130503 PL - Switzerland TA - Cerebrovasc Dis Extra JT - Cerebrovascular diseases extra JID - 101577885 PMC - PMC3656689 OID - NLM: PMC3656689 OTO - NOTNLM OT - Acute cardioembolic stroke OT - Dabigatran OT - Intracardiac thrombus OT - Nonvalvular atrial fibrillation OT - Transesophageal echocardiography EDAT- 2013/05/21 06:00 MHDA- 2013/05/21 06:01 CRDT- 2013/05/21 06:00 PHST- 2013 [ppublish] PHST- 2013/05/03 [epublish] AID - 10.1159/000351137 [doi] AID - cee-0003-0078 [pii] PST - epublish SO - Cerebrovasc Dis Extra. 2013 May 3;3(1):78-80. doi: 10.1159/000351137. Prin t 2013 Jan. PMID- 23640737 OWN - NLM STAT- MEDLINE

DA - 20130503 DCOM- 20130702 LR - 20140319 IS - 1097-0142 (Electronic) IS - 0008-543X (Linking) VI - 118 IP - 23 DP - 2012 Dec 1 TI - Late dysphagia after radiotherapy-based treatment of head and neck cancer. PG - 5793-9 LID - 10.1002/cncr.27631 [doi] AB - BACKGROUND: Changing trends in head and neck cancer (HNC) merit an underst anding of the late effects of therapy, but few studies examine dysphagia beyond 2 years of treatment. METHODS: A case series was examined to describe the pathophy siology and outcomes in dysphagic HNC survivors referred for modified barium swall ow (MBS) studies >/= 5 years after definitive radiotherapy or chemoradiothera py (January 2001 through May 2011). Functional measures included the penetration-aspiration scale (PAS), performance status scale-head and neck (PSS-HN), National Institutes of Health Swallowing Safety Scale (NIH-SSS), and MBS impairment profile (MBSImp). RESULTS: Twenty-nine patients previously treated with radiotherapy (38%) or chemoradiotherapy (62%) were included (median y ears posttreatment, 9; range, 5-19). The majority (86%) had oropharyngeal cance r; 52% were never-smokers. Seventy-five percent had T2 or T3 tumors; 52% were N+. The median age at diagnosis was 55 (range, 38-72). Abnormal late examination f indings included: dysarthria/dysphonia (76%), cranial neuropathy (48%), trismus (3 8%), and radionecrosis (10%). MBS studies confirmed pharyngeal residue and aspi ration in all dysphagic cases owing to physiologic impairment (median PAS, 8; med ian NIH-SSS, 10; median MBSImp, 18), whereas stricture was confirmed endoscopi cally in 7 (24%). Twenty-five (86%) developed pneumonia, half requiring hospitalization. Swallow postures/strategies helped 69% of cases, but no p atient achieved durable improvement across functional measures at last follow-up. Ultimately, 19 (66%) were gastrostomy-dependent. CONCLUSIONS: Although fun ctional organ preservation is commonly achieved, severe dysphagia represents a challenging late effect that may develop or progress years after radiation -based therapy for HNC. These data suggest that novel approaches are needed to mi nimize and better address this complication that is commonly refractory to many s tandard dysphagia therapies. CI - Copyright (c) 2012 American Cancer Society. FAU - Hutcheson, Katherine A AU - Hutcheson KA

AD - Department of Head and Neck Surgery, The University of Texas MD Anderson C ancer Center, Houston, Texas 77030, USA. karnold@mdanderson.org FAU - Lewin, Jan S AU - Lewin JS FAU - Barringer, Denise A AU - Barringer DA FAU - Lisec, Asher AU - Lisec A FAU - Gunn, G Brandon AU - Gunn GB FAU - Moore, Michael W S AU - Moore MW FAU - Holsinger, F Christopher AU - Holsinger FC LA - eng GR - P30 CA016672/CA/NCI NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120517 PL - United States TA - Cancer JT - Cancer JID - 0374236 SB - AIM SB - IM MH - Adult MH - Aged MH - Deglutition Disorders/diagnosis/*etiology MH - Female MH - Head and Neck Neoplasms/*radiotherapy MH - Humans MH - Male MH - Middle Aged MH - Radiation Injuries/diagnosis/*etiology MH - Retrospective Studies MH - Treatment Outcome EDAT- 2013/05/04 06:00 MHDA- 2013/07/03 06:00 CRDT- 2013/05/04 06:00 PHST- 2012/01/23 [received] PHST- 2012/03/27 [revised] PHST- 2012/04/02 [accepted] PHST- 2012/05/17 [aheadofprint] AID - 10.1002/cncr.27631 [doi] PST - ppublish SO - Cancer. 2012 Dec 1;118(23):5793-9. doi: 10.1002/cncr.27631. Epub 2012 May 17. PMIDOWN STATDA DCOMLR IS IS VI IP DP 23559290 NLM MEDLINE 20130405 20130930 20131112 0717-6163 (Electronic) 0034-9887 (Linking) 140 10 2012 Oct

TI - [Posterior reversible encephalopathy as the first manifestation of Guillai n-Barre syndrome. Report of one case]. PG - 1316-20 LID - 10.4067/S0034-98872012001000012 [doi] LID - S0034-98872012001000012 [pii] AB - BACKGROUND: We report a 56 year old male hypertensive, who presented with a posterior reversible encephalopathy syndrome (PRES) as an initial manifest ation of Guillain-Barre syndrome (GBS). His first symptoms were right hemiparesi s and hemihypoesthesia, followed by headache, dizziness, dysarthria and a genera l feeling of discomfort. On the third day, flaccid tetraparesis, impairment of consciousness, epileptic seizures and respiratory failure appeared, along with severe hypertension. Cerebral Magnetic Resonance Imaging showed the characteristic PRES lesions. Cerebrospinal fluid analyses revealed albumin-cytological dissociation and nerve conduction studies showed an ax onal demyelinating polyradiculoneuropathy, which confirmed the diagnosis of GBS . Treatment with intravenous immunoglobulin was given together with antihypertensive therapy and mechanical ventilation, achieving an importan t clinical and imaging remission of PRES, but maintaining tetraparesis durin g the hospitalization. Twelve months after discharge and regular motor rehabilit ation, the patient achieved complete autonomy on the activities of daily living. It has been postulated that the autonomic failure and the elevation of circulatin g pro-inflammatory cytokines in GBS may be the cause of a breach in the bloo d-brain barrier, thus causing PRES, that can completely remit with an adequate management. FAU - Urrutia L, Sergio AU - Urrutia L S AD - Unidad de Neurologia Adultos, Hospital Clinico FUSAT, Rancagua, Chile. drsergiourrutia@gmail.com FAU - Venegas R, Eduardo AU - Venegas R E FAU - Figueroa V, Cristian AU - Figueroa V C FAU - Carrizo C, Catalina AU - Carrizo C C LA - spa PT - Case Reports PT - English Abstract PT - Journal Article TT - Encefalopatia posterior reversible como primera manifestacion del Sindrome de Guillain-Barre PL - Chile TA - Rev Med Chil JT - Revista medica de Chile JID - 0404312

SB MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTPHSTAID AID PST SO 12. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI le

IM Diagnosis, Differential Guillain-Barre Syndrome/*complications/diagnosis Humans Hypertension/*complications Magnetic Resonance Imaging Male Middle Aged Posterior Leukoencephalopathy Syndrome/diagnosis/*etiology 2013/04/06 06:00 2013/10/01 06:00 2013/04/06 06:00 2011/03/14 [received] 2011/05/17 [accepted] S0034-98872012001000012 [pii] 10.4067/S0034-98872012001000012 [doi] ppublish Rev Med Chil. 2012 Oct;140(10):1316-20. doi: 10.4067/S0034-988720120010000 23475537 NLM MEDLINE 20130430 20130617 20131121 1465-3621 (Electronic) 0368-2811 (Linking) 43 5 2013 May Anti-Yo antibody-mediated paraneoplastic cerebellar degeneration in a fema

patient with pleural malignant mesothelioma. PG - 563-8 LID - 10.1093/jjco/hyt031 [doi] AB - Paraneoplastic cerebellar degeneration is a rare non-metastatic complicati on of malignancies. It presents with acute or subacute onset of ataxia, dysarthr ia and intention tremor. Paraneoplastic cerebellar degeneration is most commonly associated with malignancies of the ovary, breast and lung. The anti-Yo (anti-Purkinje cells) antibodies that specifically damage the Purkinje cel ls of the cerebellum are found in the serum and cerebrospinal fluid. Anti-Yo-rel ated paraneoplastic cerebellar degeneration is most commonly found in women wit h gynecological and breast cancers, but it is reported in other malignancies . Patients with paraneoplastic syndromes most often present with neurologic symptoms before an underlying cancer is detected. We report a case of anti-Yo-related paraneoplastic cerebellar degeneration associated with ple ural malignant mesothelioma in a 51-year-old female patient. She presented to o ur department with a 2-week history after the last chemotherapy of progressiv e dizziness related to head movement, nausea, vomiting, ataxia and unsteady gait. A

western blot assay was negative for anti-Hu, anti-Ri, anti-Ma2, anti-CV2 a nd anti-amphiphysin paraneoplastic antibody markers but positive for anti-Yo. In conclusion, we report a case of paraneoplastic cerebellar degeneration in a patient with pleural malignant mesothelioma because of the rarity of this neurologic presentation after the diagnosis of malignant mesothelioma and of the association with anti-Yo antibodies. FAU - Tanriverdi, Ozgur AU - Tanriverdi O AD - Department of Medical Oncology, Mugla Sitki Kocman University Education an d Research Hospital, Mugla, Turkey. ozgurtanriverdi@hotmail.com FAU - Meydan, Nezih AU - Meydan N FAU - Barutca, Sabri AU - Barutca S FAU - Ozsan, Nazan AU - Ozsan N FAU - Gurel, Duygu AU - Gurel D FAU - Veral, Ali AU - Veral A LA - eng PT - Case Reports PT - Journal Article DEP - 20130308 PL - England TA - Jpn J Clin Oncol JT - Japanese journal of clinical oncology JID - 0313225 RN - 0 (CDR2 protein, human) RN - 0 (Nerve Tissue Proteins) SB - IM MH - Female MH - Humans MH - Mesothelioma/*complications/diagnosis MH - Middle Aged MH - Multimodal Imaging MH - Nerve Tissue Proteins/*immunology MH - Paraneoplastic Cerebellar Degeneration/*diagnosis/drug therapy/*immunology MH - Pleural Neoplasms/*complications/diagnosis MH - Positron-Emission Tomography MH - Tomography, X-Ray Computed EDAT- 2013/03/12 06:00 MHDA- 2013/06/19 06:00 CRDT- 2013/03/12 06:00 PHST- 2013/03/08 [aheadofprint] AID - hyt031 [pii] AID - 10.1093/jjco/hyt031 [doi] PST - ppublish SO - Jpn J Clin Oncol. 2013 May;43(5):563-8. doi: 10.1093/jjco/hyt031. Epub 201 3 Mar 8. PMID- 23468819 OWN - NLM STAT- MEDLINE

DA DCOMLR IS IS VI IP DP TI -

20130307 20131122 20131203 1866-0452 (Electronic) 1866-0452 (Linking) 110 7 2013 Feb Who receives rehabilitation after stroke?: Data from the quality assurance project "Stroke Register Northwest Germany". PG - 101-7 LID - 10.3238/arztebl.2013.0101 [doi] AB - BACKGROUND: Neurological rehabilitation after stroke lowers rates of death , dependency, and institutionalization. Little research has yet addressed th e factors affecting the selection of ischemic stroke patients for rehabilita tive treatment. METHOD: The database for this study consisted of all cases of i schemic stroke (ICD-10 code I63) that occurred in 2010 and 2011 in the neurologica l inpatient care facilities participating in the "Stroke Register Northwest Germany" quality assurance project. A primary target group for rehabilitat ion was defined a priori (Barthel Index at discharge /= pound25,000 to gain one unit of utility. CONCLUSIONS: These findings exclude the possibil ity of a clinically significant difference of 0.5 points on the TOM. There was no evidence, on any measure, of added benefit of early communication therapy beyond that from AC. It is unclear whether therapy is more or less cost-effective than AC. Early, frequent contact was highly valued by users and had good uptake . Functional communication improved for both groups, plausibly due to natura l recovery and early and regular opportunity to practise everyday communicat ion with a professional (therapist/visitor). There is no evidence to recommend enhancing the provision of early communication therapy by a qualified SL therapist over and above usual care. SL therapy service reorganisation sho uld consider skill mix and timing within a stepped care model and should take place

within the context of a trial. FAU - Bowen, A AU - Bowen A AD - University of Manchester MAHSC (Manchester Academic Health Science Centre) , Manchester, UK. audrey.bowen@manchester.ac.uk FAU - Hesketh, A AU - Hesketh A FAU - Patchick, E AU - Patchick E FAU - Young, A AU - Young A FAU - Davies, L AU - Davies L FAU - Vail, A AU - Vail A FAU - Long, A AU - Long A FAU - Watkins, C AU - Watkins C FAU - Wilkinson, M AU - Wilkinson M FAU - Pearl, G AU - Pearl G FAU - Lambon Ralph, M AU - Lambon Ralph M FAU - Tyrrell, P AU - Tyrrell P CN - ACT NoW investigators LA - eng SI - ISRCTN/ISRCTN78617680 PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - Health Technol Assess JT - Health technology assessment (Winchester, England) JID - 9706284 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Aphasia/therapy MH - Dysarthria/therapy MH - Feasibility Studies MH - Female MH - Great Britain MH - Humans MH - Interviews as Topic MH - Male MH - Middle Aged MH - Outcome Assessment (Health Care)/*methods MH - *Patient Satisfaction MH - Speech Therapy/*economics MH - State Medicine MH - Stroke/physiopathology/*rehabilitation EDAT- 2012/05/23 06:00 MHDA- 2012/10/19 06:00 CRDT- 2012/05/23 06:00 AID - 10.3310/hta16260 [doi]

PST - ppublish SO - Health Technol Assess. 2012 May;16(26):1-160. doi: 10.3310/hta16260. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB lly we describe the case of a 46-year-old patient with metastatic rectal carcinom a who received second-line therapy with irinotecan and developed isolated transi ent dysarthria (with normal MR imaging of the brain) following each administra tion of irinotecan. Neurological and logopedical evaluation revealed that the dysa rthria predominantly resulted from a reduced capacity in fine-tuning of motor fun ctions of the tip of the tongue and a minimal reduction in the power of speech at labiodental contact. As hypoglossal nerve activity has been reported to be especially susceptible to cholinergic stimulation and irinotecan can cause cholinergic side effects by binding to and inactivating acetylcholinestera se, we suspect this mechanism to be responsible for irinotecan-induced dysarthria . FAU - Dressel, Albertine J AU - Dressel AJ AD - Department of Medical Oncology, VU University Medical Center, Amsterdam, T he Netherlands. FAU - van der Mijn, Johannes C AU - van der Mijn JC FAU - Aalders, Ijke J AU - Aalders IJ FAU - Rinkel, Rico N P M AU - Rinkel RN FAU - van der Vliet, Hans J AU - van der Vliet HJ LA - eng PT - Journal Article DEP - 20120118 PL - Switzerland TA - Case Rep Oncol JT - Case reports in oncology JID - 101517601 PMC - PMC3290033 OID - NLM: PMC3290033 22379477 NLM PubMed-not-MEDLINE 20120301 20121002 20130529 1662-6575 (Electronic) 1662-6575 (Linking) 5 1 2012 Jan Irinotecan-induced dysarthria. 47-51 10.1159/000336156 [doi] Colorectal carcinomas are among the most common tumor types and are genera treated with palliative chemotherapy in case of metastatic disease. Here,

OTO OT OT OT EDATMHDACRDTPHSTAID AID PST SO 18.

NOTNLM Colorectal cancer Dysarthria Irinotecan 2012/03/02 06:00 2012/03/02 06:01 2012/03/02 06:00 2012/01/18 [epublish] 000336156 [doi] cro-0005-0047 [pii] ppublish Case Rep Oncol. 2012 Jan;5(1):47-51. doi: 10.1159/000336156. Epub 2012 Jan

PMID- 22295152 OWN - NLM STAT- MEDLINE DA - 20120201 DCOM- 20120524 LR - 20131014 IS - 1936-2625 (Electronic) IS - 1936-2625 (Linking) VI - 5 IP - 1 DP - 2012 TI - Rare case of a primary non-dural central nervous system low grade B-cell l ymphoma and literature review. PG - 89-95 AB - We present a case of a 70-year-old HIV negative man with a five-year histo ry of progressive dysnomia and new onset right extremity numbness, dysarthria, a nd blurry vision. On magnetic resonance imaging (MRI), an infiltrative enhanc ing tumor was noted. Follow up brain biopsy results revealed a small lymphocyt ic infiltrate with scattered plasma cells in a predominantly perivascular gro wth pattern. Flow-cytometric findings revealed a lambda monotypic B-cell popul ation. The morphology and the flow cytometric findings were consistent with invol vement by a low grade B-cell lymphoma. Subsequent positron emission tomography (P ET) studies along with bone marrow biopsy and serum protein electrophoresis sh owed no evidence of systemic disease. The above findings are consistent with invol vement by a non-dural extranodal marginal zone B-cell lymphoma (MZBCL) primary to the central nervous system (CNS). This is the first reported case of a primary CNS MZBCL with flow cytometric analysis. A review of literature on this rare e ntity is also included. FAU - Papanicolau-Sengos, Antonios AU - Papanicolau-Sengos A AD - Department of Pathology, University of California San Diego School of Medi cine

and Veteran Administration Medical Center of San Diego, 9500 Gilman Drive # 9113, La Jolla, CA 92093-9113, USA. FAU - Wang-Rodriguez, Jessica AU - Wang-Rodriguez J FAU - Wang, Huan-You AU - Wang HY FAU - Lee, Roland R AU - Lee RR FAU - Wong, Anna AU - Wong A FAU - Hansen, Lawrence A AU - Hansen LA FAU - Mahooti, Sepi AU - Mahooti S FAU - Rashidi, Hooman H AU - Rashidi HH LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20120101 PL - United States TA - Int J Clin Exp Pathol JT - International journal of clinical and experimental pathology JID - 101480565 SB - IM MH - Aged MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Central Nervous System Neoplasms/drug therapy/*pathology/physiopathology MH - Flow Cytometry MH - Humans MH - Lymphoma, B-Cell, Marginal Zone/drug therapy/*pathology/physiopathology MH - Male MH - Neoplasm Grading PMC - PMC3267491 OID - NLM: PMC3267491 OTO - NOTNLM OT - Primary Non-Dural Central Nervous System Low grade B-cell lymphoma OT - extranodal marginal zone lymphoma EDAT- 2012/02/02 06:00 MHDA- 2012/05/25 06:00 CRDT- 2012/02/02 06:00 PHST- 2011/11/20 [received] PHST- 2011/12/07 [accepted] PHST- 2012/01/01 [epublish] PST - ppublish SO - Int J Clin Exp Pathol. 2012;5(1):89-95. Epub 2012 Jan 1. PMIDOWN STATDA DCOMLR IS IS VI IP DP 22271664 NLM MEDLINE 20120227 20120409 20140319 1460-2156 (Electronic) 0006-8950 (Linking) 135 Pt 3 2012 Mar

TI PG LID AB d by of 2-3

A proposed staging system for amyotrophic lateral sclerosis. 847-52 10.1093/brain/awr351 [doi] Amyotrophic lateral sclerosis is a neurodegenerative disorder characterize progressive loss of upper and lower motor neurons, with a median survival

years. Although various phenotypic and research diagnostic classification systems exist and several prognostic models have been generated, there is no stagi ng system. Staging criteria for amyotrophic lateral sclerosis would help to p rovide a universal and objective measure of disease progression with benefits for patient care, resource allocation, research classifications and clinical t rial design. We therefore sought to define easily identified clinical milestone s that could be shown to occur at specific points in the disease course, reflect disease progression and impact prognosis and treatment. A tertiary referral centre clinical database was analysed, consisting of 1471 patients with amyotroph ic lateral sclerosis seen between 1993 and 2007. Milestones were defined as s ymptom onset (functional involvement by weakness, wasting, spasticity, dysarthria or dysphagia of one central nervous system region defined as bulbar, upper li mb, lower limb or diaphragmatic), diagnosis, functional involvement of a secon d region, functional involvement of a third region, needing gastrostomy and non-invasive ventilation. Milestone timings were standardized as proportio ns of time elapsed through the disease course using information from patients wh o had died by dividing time to a milestone by disease duration. Milestones occur red at predictable proportions of the disease course. Diagnosis occurred at 35% t hrough the disease course, involvement of a second region at 38%, a third region at 61%, need for gastrostomy at 77% and need for non-invasive ventilation at 80%. We therefore propose a simple staging system for amyotrophic lateral sclerosi s. Stage 1: symptom onset (involvement of first region); Stage 2A: diagnosis; Stage 2B: involvement of second region; Stage 3: involvement of third region; St age 4A: need for gastrostomy; and Stage 4B: need for non-invasive ventilation. Val idation of this staging system will require further studies in other populations, in population registers and in other clinic databases. The standardized times to milestones may well vary between different studies and populations, althou gh the stages themselves and their meanings are likely to remain unchanged. FAU - Roche, Jose C

AU - Roche JC AD - MRC Centre for Neurodegeneration Research, King's College London, Institut e of Psychiatry, London SE5 8AF, UK. FAU - Rojas-Garcia, Ricardo AU - Rojas-Garcia R FAU - Scott, Kirsten M AU - Scott KM FAU - Scotton, William AU - Scotton W FAU - Ellis, Catherine E AU - Ellis CE FAU - Burman, Rachel AU - Burman R FAU - Wijesekera, Lokesh AU - Wijesekera L FAU - Turner, Martin R AU - Turner MR FAU - Leigh, P Nigel AU - Leigh PN FAU - Shaw, Christopher E AU - Shaw CE FAU - Al-Chalabi, Ammar AU - Al-Chalabi A LA - eng GR - 089701/Wellcome Trust/United Kingdom GR - G0500289/Medical Research Council/United Kingdom GR - G0701923/Medical Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120123 PL - England TA - Brain JT - Brain : a journal of neurology JID - 0372537 SB - AIM SB - IM MH - Age of Onset MH - Aged MH - Amyotrophic Lateral Sclerosis/classification/diagnosis/*pathology MH - Cohort Studies MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Gastrostomy MH - Humans MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Models, Statistical MH - Patient Selection MH - Prognosis MH - Reproducibility of Results MH - Respiration, Artificial MH - Sex Factors MH - Survival Analysis PMC - PMC3286327 OID - NLM: PMC3286327 EDAT- 2012/01/25 06:00 MHDA- 2012/04/10 06:00

CRDTPHSTAID AID PST SO 23. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB resis

2012/01/25 06:00 2012/01/23 [aheadofprint] awr351 [pii] 10.1093/brain/awr351 [doi] ppublish Brain. 2012 Mar;135(Pt 3):847-52. doi: 10.1093/brain/awr351. Epub 2012 Jan 22247979 NLM MEDLINE 20120806 20121210 20131121 2000-1967 (Electronic) 0300-9734 (Linking) 117 3 2012 Aug A case of hypoglycemic hemiparesis and literature review. 347-51 10.3109/03009734.2011.652748 [doi] An 89-year-old man with diabetes treated with metformin 500 mg/day and glimepiride 4 mg/day was hospitalized because of hypoglycemic right hemipa and dysarthria (casual glucose value 1.8 mmol/L), which resolved quickly following administration of 40 mL of 40% dextrose. Hemiparesis is a rare s

ymptom (4.2%) of hypoglycemia. There are about 200 case reports of hypoglycemic hemiparesis. The average glucose level at which hemiparesis developed was 1.8 mmol/L. Right-sided hemiparesis predominated (R 66%; L 34%). On imaging st udies, abnormal findings were frequently observed in the internal capsule or sple nium of the corpus callosum. The mechanism of hemiparesis is not fully understood. The existence of cases in which hypoglycemia cannot be distinguished from stro ke on imaging studies suggests the importance of measurement of the blood glucos e level when the symptoms of stroke are first recognized. FAU - Yoshino, Tetsuhiro AU - Yoshino T AD - Division of Endocrinology, Metabolism and Nephrology Department of Interna l Medicine Keio University, School of Medicine, Shinjuku, Japan. tetta213@yahoo.co.jp FAU - Meguro, Shu AU - Meguro S FAU - Soeda, Yukie AU - Soeda Y FAU - Itoh, Arata AU - Itoh A FAU - Kawai, Toshihide AU - Kawai T FAU - Itoh, Hiroshi AU - Itoh H LA - eng PT - Case Reports

PT PT DEP PL TA JT JID RN RN RN RN SB MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDACRDTPHSTAID PST SO Epub PMIDOWN STATDA DCOMIS IS VI IP DP TI onian

Journal Article Review 20120117 England Ups J Med Sci Upsala journal of medical sciences 0332203 0 (Blood Glucose) 0 (Sulfonylurea Compounds) 9100L32L2N (Metformin) 93479-97-1 (glimepiride) IM Aged Aged, 80 and over Blood Glucose/analysis Diabetes Mellitus/drug therapy Humans Hypoglycemia/*complications Male Metformin/administration & dosage/therapeutic use Paresis/*diagnosis/etiology Sulfonylurea Compounds/administration & dosage/therapeutic use PMC3410296 NLM: PMC3410296 2012/01/18 06:00 2012/12/12 06:00 2012/01/18 06:00 2012/01/17 [aheadofprint] 10.3109/03009734.2011.652748 [doi] ppublish Ups J Med Sci. 2012 Aug;117(3):347-51. doi: 10.3109/03009734.2011.652748. 2012 Jan 17. 22142042 NLM MEDLINE 20111206 20120403 2146-8427 (Electronic) 1304-0855 (Linking) 9 6 2011 Dec Transient improvement of acquired hepatocerebral degeneration with parkins

symptoms after failed liver transplant: case report and literature review. PG - 363-9 AB - OBJECTIVES: Acquired (non-Wilsonian) hepato-cerebral degeneration is an infrequent neurologic disorder in patients with liver dysfunction and longstanding portal-systemic shunting. The clinical manifestations include dysarthria, ataxia, tremor, and cognitive dysfunction. Typically, patients with acquired hepatocerebral degeneration respond poorly to medical therapy as the underlying end-stage liver disease remains. Information regarding the effe ct of orthotopic liver transplant on acquired hepatocerebral degeneration, howev er, is limited and conflicting. MATERIALS AND METHODS: We conducted a review of

literature via a PubMed search to summarize the effect of orthotopic liver transplant on acquired hepatocerebral degeneration. RESULTS: We present a 56-year-old man with compensated hepatitis C cirrhosis who developed acqui red hepatocerebral degeneration with Parkinsonian symptoms refractory to conve ntional Parkinson medical therapy. Orthotopic liver transplant led to marked clini cal improvement of the acquired hepatocerebral degeneration. However, the pati ent developed recurrence of acquired hepatocerebral degeneration 6-week postorthotopic liver transplant as he developed graft failure from aggress ive progressive hepatitis C recurrence. Our review found a heterogeneous group of case series, suggesting that the experience with orthotopic liver transpla nt is variable. CONCLUSIONS: Our experience demonstrates that orthotopic liver transplant may lead to resolution of acquired hepatocerebral degeneration; however, acquired hepatocerebral degeneration may return with recurrent li ver disease. Future studies with long-term follow-up are needed. FAU - Chen, Yuanyuan AU - Chen Y AD - Department of Medicine, University of British Columbia, Vancouver, BC, Can ada. FAU - Haque, Mazhar AU - Haque M FAU - Yoshida, Eric M AU - Yoshida EM LA - eng PT - Case Reports PT - Journal Article PT - Review PL - Turkey TA - Exp Clin Transplant JT - Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation JID - 101207333 RN - 0 (Antiparkinson Agents) RN - 0 (Antiviral Agents) SB - IM MH - Antiparkinson Agents/therapeutic use MH - Antiviral Agents/therapeutic use MH - End Stage Liver Disease/etiology/*surgery MH - Fatal Outcome MH - Graft Rejection/*etiology MH - Hepatitis C/*complications/diagnosis/drug therapy MH - Hepatolenticular Degeneration/diagnosis/etiology/*surgery MH - Humans MH - Liver Cirrhosis/etiology/*surgery MH - Liver Transplantation/*adverse effects MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Parkinsonian Disorders/diagnosis/drug therapy/etiology/*surgery MH - Recurrence MH - Treatment Failure EDAT- 2011/12/07 06:00

MHDACRDTPST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI PG AB cally in 180

2012/04/04 06:00 2011/12/07 06:00 ppublish Exp Clin Transplant. 2011 Dec;9(6):363-9. 22123476 NLM MEDLINE 20111129 20120425 1349-8029 (Electronic) 0470-8105 (Linking) 51 11 2011 Complications of subthalamic nucleus stimulation in Parkinson's disease. 749-55 Subthalamic nucleus deep brain stimulation (STN-DBS) is effective for medi refractory Parkinson's disease. We retrospectively analyzed complications consecutive patients who underwent bilateral STN-DBS. Surgery-related complications were symptomatic intracerebral hemorrhage in 2, chronic subd

ural hematoma in 1, and transient deterioration of medication-induced psychosis in 2 patients. Device-related complications involved device infection in 5, ski n erosion in 5, and implantable pulse generator malfunction in 2 patients. A ll of these patients required surgical repair. Surgery and device-related compli cations could be reduced with increased surgical experience and the introduction o f new surgical equipment and technology. Treatment or stimulation-related compli cations were intractable dyskinesia/dystonia in 11, problematic dysarthria in 7, a praxia of eyelid opening (ALO) in 11, back pain in 10, and restless leg syndrome in 6 patients. Neuropsychiatric complications were transient mood changes in so me, impulse control disorder in 2, severe depression related to excessive redu ction of dopaminergic medications in 2, rapid progression of dementia in 1, and suicide attempts in 2 patients. Most complications were mild and transient. Dysart hria and ALO were the most frequent permanent sequelae after STN-DBS. Treatment-related adverse events may be caused not only by the effect of stimulation effect but also excessive reduction of dopaminergic medication , or progression of the disease. In conclusion, STN-DBS seems to be a relativel y safe procedure. Although serious complications with permanent sequelae are rare , significant incidences of adverse effects occur. Physicians engaged in thi s treatment should have a comprehensive understanding of the probable compli cations

and how to avoid them. FAU - Umemura, Atsushi AU - Umemura A AD - Department of Neurosurgery, Nagoya City University Graduate School of Medi cine, Aichi. aume@med.nagoya-cu.ac.jp FAU - Oka, Yuichi AU - Oka Y FAU - Yamamoto, Kenichi AU - Yamamoto K FAU - Okita, Kenji AU - Okita K FAU - Matsukawa, Noriyuki AU - Matsukawa N FAU - Yamada, Kazuo AU - Yamada K LA - eng PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Apraxias/*etiology/pathology MH - Cohort Studies MH - Deep Brain Stimulation/*adverse effects MH - Dysarthria/*etiology MH - Dyskinesias/*etiology MH - Eyelid Diseases/etiology/pathology MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Mental Disorders/etiology MH - Middle Aged MH - Parkinson Disease/*therapy MH - Retrospective Studies EDAT- 2011/11/30 06:00 MHDA- 2012/04/26 06:00 CRDT- 2011/11/30 06:00 AID - JST.JSTAGE/nmc/51.749 [pii] PST - ppublish SO - Neurol Med Chir (Tokyo). 2011;51(11):749-55. PMID- 22078654 OWN - NLM STAT- MEDLINE DA - 20130507 DCOM- 20131219 IS - 1578-1968 (Electronic) IS - 0213-4853 (Linking) VI - 28 IP - 4 DP - 2013 May TI - Acute persistent dysarthria and dizziness with previous neurological study : is it enough to establish a diagnosis?

PG LID LID FAU AU FAU AU FAU AU FAU AU LA LA PT PT DEP PL TA JT JID RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTPHSTPHSTPHSTAID AID PST SO 1 Nov PMIDOWN STATDA DCOMLR IS IS VI IP -

250-2 10.1016/j.nrl.2011.09.001 [doi] S0213-4853(11)00394-X [pii] Fernandez Dominguez, J Fernandez Dominguez J Garcia Rodriguez, R Garcia Rodriguez R Vega, P Vega P Calleja Puerta, S Calleja Puerta S eng spa Case Reports Letter 20111109 Spain Neurologia Neurologia (Barcelona, Spain) 9005460 0 (Adrenergic beta-Antagonists) IM Adrenergic beta-Antagonists/therapeutic use Cerebral Angiography Dizziness/*diagnosis/*etiology/ultrasonography Dysarthria/*diagnosis/*etiology/ultrasonography Gait Disorders, Neurologic/diagnosis/etiology Humans Hypertension/complications/drug therapy Magnetic Resonance Angiography Male Middle Aged Nervous System Diseases/*complications/*diagnosis/ultrasonography Neurologic Examination Smoking/adverse effects Ultrasonography, Doppler, Transcranial 2011/11/15 06:00 2013/12/20 06:00 2011/11/15 06:00 2011/05/19 [received] 2011/09/12 [revised] 2011/09/15 [accepted] 2011/11/09 [aheadofprint] S0213-4853(11)00394-X [pii] 10.1016/j.nrl.2011.09.001 [doi] ppublish Neurologia. 2013 May;28(4):250-2. doi: 10.1016/j.nrl.2011.09.001. Epub 201 9. 22022010 NLM PubMed-not-MEDLINE 20111024 20111110 20130529 1998-3751 (Electronic) 0253-7613 (Linking) 43 5

DP - 2011 Sep TI - Quadriparesis and dysarthria due to tetrabenazine therapy in a child with rheumatic chorea. PG - 601-2 LID - 10.4103/0253-7613.84982 [doi] AB - Tetrabenazine (TBZ) is widely used to treat hyperkinetic movement disorder s in adults; however, published experience with the drug in children is limited . Common side effects of TBZ include drowsiness, sedation, weakness, Parkins onism, depression, and acute akathisia, all of which are reversible with decrease d doses. We report here a 7-year-old girl with rheumatic chorea who develope d acute akinesia of all four limbs and dysarthria due to TBZ therapy. Withdrawal o f the drug led to rapid improvement within 18 hours. FAU - Zaki, Syed Ahmed AU - Zaki SA AD - Department of Pediatrics, Lokmanya Tilak Municipal General Hospital and Me dical College, Sion, Mumbai - 400 022, India. FAU - Lad, Vijay AU - Lad V FAU - Shanbag, Preeti AU - Shanbag P LA - eng PT - Journal Article PL - India TA - Indian J Pharmacol JT - Indian journal of pharmacology JID - 7902477 PMC - PMC3195137 OID - NLM: PMC3195137 OTO - NOTNLM OT - Dysarthria OT - parkinsonism OT - quadriparesis OT - rheumatic chorea OT - tetrabenazine EDAT- 2011/10/25 06:00 MHDA- 2011/10/25 06:01 CRDT- 2011/10/25 06:00 PHST- 2011/01/28 [received] PHST- 2011/02/16 [revised] PHST- 2011/07/01 [accepted] AID - 10.4103/0253-7613.84982 [doi] AID - IJPharm-43-601 [pii] PST - ppublish SO - Indian J Pharmacol. 2011 Sep;43(5):601-2. doi: 10.4103/0253-7613.84982. PMIDOWN STATDA DCOMLR IS IS 22004192 NLM MEDLINE 20111129 20120413 20131212 1939-1277 (Electronic) 0096-1523 (Linking)

VI IP DP TI PG LID AB tive

37 6 2011 Dec Word recognition reflects dimension-based statistical learning. 1939-56 10.1037/a0025641 [doi] Speech processing requires sensitivity to long-term regularities of the na

language yet demands listeners to flexibly adapt to perturbations that ari se from talker idiosyncrasies such as nonnative accent. The present experiments investigate whether listeners exhibit dimension-based statistical learning of correlations between acoustic dimensions defining perceptual space for a g iven speech segment. While engaged in a word recognition task guided by a perce ptually unambiguous voice-onset time (VOT) acoustics to signal beer, pier, deer, o r tear, listeners were exposed incidentally to an artificial "accent" deviating fr om English norms in its correlation of the pitch onset of the following vowel (F0) to VOT. Results across four experiments are indicative of rapid, dimension -based statistical learning; reliance on the F0 dimension in word recognition was rapidly down-weighted in response to the perturbation of the correlation b etween F0 and VOT dimensions. However, listeners did not simply mirror the shortterm input statistics. Instead, response patterns were consistent with a linger ing influence of sensitivity to the long-term regularities of English. This su ggests that the very acoustic dimensions defining perceptual space are not fixed and, rather, are dynamically and rapidly adjusted to the idiosyncrasies of loca l experience, such as might arise from nonnative-accent, dialect, or dysarth ria. The current findings extend demonstrations of "object-based" statistical l earning across speech segments to include incidental, online statistical learning of regularities residing within a speech segment. FAU - Idemaru, Kaori AU - Idemaru K AD - Department of East Asian Languages and Literatures, University of Oregon, Eugene, OR 97403, USA. idemaru@uoregon.edu FAU - Holt, Lori L AU - Holt LL LA - eng GR - R01 DC004674/DC/NIDCD NIH HHS/United States GR - R01 DC004674-11/DC/NIDCD NIH HHS/United States GR - R01DC004674/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S.

DEP - 20111017 PL - United States TA - J Exp Psychol Hum Percept Perform JT - Journal of experimental psychology. Human perception and performance JID - 7502589 SB - IM MH - Acoustic Stimulation MH - Humans MH - *Learning MH - Psycholinguistics MH - Speech MH - *Speech Perception MH - Time Factors PMC - PMC3285244 MID - NIHMS356161 OID - NLM: NIHMS356161 OID - NLM: PMC3285244 EDAT- 2011/10/19 06:00 MHDA- 2012/04/14 06:00 CRDT- 2011/10/19 06:00 PHST- 2011/10/17 [aheadofprint] AID - 2011-23770-001 [pii] AID - 10.1037/a0025641 [doi] PST - ppublish SO - J Exp Psychol Hum Percept Perform. 2011 Dec;37(6):1939-56. doi: 10.1037/a0 025641. Epub 2011 Oct 17. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI 21976947 NLM MEDLINE 20111006 20120202 20131016 1011-8942 (Print) 1011-8942 (Linking) 25 5 2011 Oct Ophthalmic artery obstruction and cerebral infarction following periocular injection of autologous fat. PG - 358-61 LID - 10.3341/kjo.2011.25.5.358 [doi] AB - We report a case of ophthalmic artery obstruction combined with brain infa rction following periocular autologous fat injection. The patient, a 44-year-old woman, visited our hospital for decreased visual acuity in her left eye and dysar thria one hour after receiving an autologous fat injection in the periocular are a. Her best corrected visual acuity for the concerned eye was no light perception . Also, a relative afferent pupillary defect was detected in this eye. The left fu ndus exhibited widespread retinal whitening with visible emboli in several reti nal arterioles. Diffusion-weighted magnetic resonance imaging of the brain sho wed a hyperintense lesion at the left insular cortex. Therefore, we diagnosed

ophthalmic artery obstruction and left middle cerebral artery infarction d ue to fat emboli. The patient was managed with immediate ocular massage, carbon dioxide, and oxygen therapy. Following treatment, dysarthria improved considerably but there was no improvement in visual acuity. FAU - Lee, Chang Mok AU - Lee CM AD - Department of Ophthalmology, Kangdong Sacred Heart Hospital, Hallym Univer sity College of Medicine, Seoul, Korea. FAU - Hong, In Hwan AU - Hong IH FAU - Park, Sung Pyo AU - Park SP LA - eng PT - Case Reports PT - Journal Article DEP - 20110920 PL - Korea (South) TA - Korean J Ophthalmol JT - Korean journal of ophthalmology : KJO JID - 8804513 SB - IM MH - Adult MH - Arterial Occlusive Diseases/diagnosis/*etiology MH - Female MH - Fluorescein Angiography MH - Follow-Up Studies MH - Fundus Oculi MH - Humans MH - Infarction, Middle Cerebral Artery/*complications/diagnosis MH - Magnetic Resonance Imaging MH - *Ophthalmic Artery MH - Orbit MH - Subcutaneous Fat/*transplantation MH - Transplantation, Autologous/adverse effects MH - Visual Acuity PMC - PMC3178774 OID - NLM: PMC3178774 OTO - NOTNLM OT - Abdominal fat OT - Cerebral infarction OT - Ophthalmic artery OT - Retinal artery occlusion EDAT- 2011/10/07 06:00 MHDA- 2012/02/03 06:00 CRDT- 2011/10/07 06:00 PHST- 2010/04/28 [received] PHST- 2010/08/16 [accepted] PHST- 2011/09/20 [epublish] AID - 10.3341/kjo.2011.25.5.358 [doi] PST - ppublish SO - Korean J Ophthalmol. 2011 Oct;25(5):358-61. doi: 10.3341/kjo.2011.25.5.358 . Epub 2011 Sep 20. PMIDOWN STATDA 21969916 NLM PubMed-not-MEDLINE 20111004

DCOM- 20111110 LR - 20120507 IS - 2042-0080 (Electronic) IS - 2042-0080 (Linking) VI - 2011 DP - 2011 TI - Assessment of prosodic communicative efficiency in Parkinson's disease as judged by professional listeners. PG - 129310 LID - 10.4061/2011/129310 [doi] AB - This study examines the impact of Parkinson's disease (PD) on communicativ e efficiency conveyed through prosody. A new assessment method for evaluatin g productive prosodic skills in Dutch speaking dysarthric patients was devis ed and tested on 36 individuals (18 controls, 18 PD patients). Three professional listeners judged the intended meanings in four communicative functions of Dutch prosody: Boundary Marking, Focus, Sentence Typing, and Emotional Prosody. Each function was tested through reading and imitation. Interrater agreement wa s calculated. Results indicated that healthy speakers, compared to PD patien ts, performed significantly better on imitation of Boundary Marking, Focus, an d Sentence Typing. PD patients with a moderate or severe dysarthria performe d significantly worse on imitation of Focus than on reading of Focus. No significant differences were found for Emotional Prosody. Judges agreed we ll on all tasks except Emotional Prosody. Future research will focus on elaborat ing the assessment and on developing a therapy programme paralleling the assessmen t. FAU - Martens, Heidi AU - Martens H AD - Rehabilitation Centre for Communication Disorders, Antwerp University Hosp ital, Wilrijkstraat 10, 2650 Edegem, Belgium. FAU - Van Nuffelen, Gwen AU - Van Nuffelen G FAU - Cras, Patrick AU - Cras P FAU - Pickut, Barbara AU - Pickut B FAU - De Letter, Miet AU - De Letter M FAU - De Bodt, Marc AU - De Bodt M LA - eng PT - Journal Article DEP - 20110928 PL - United States TA - Parkinsons Dis JT - Parkinson's disease JID - 101539877 PMC - PMC3182398

OID EDATMHDACRDTPHSTPHSTPHSTPHSTAID PST SO 28.

NLM: PMC3182398 2011/10/05 06:00 2011/10/05 06:01 2011/10/05 06:00 2011/04/01 [received] 2011/07/03 [revised] 2011/07/23 [accepted] 2011/09/28 [epublish] 10.4061/2011/129310 [doi] ppublish Parkinsons Dis. 2011;2011:129310. doi: 10.4061/2011/129310. Epub 2011 Sep

PMID- 21953693 OWN - NLM STAT- MEDLINE DA - 20111123 DCOM- 20120405 LR - 20131016 IS - 1531-8257 (Electronic) IS - 0885-3185 (Linking) VI - 26 IP - 13 DP - 2011 Nov TI - Treatment of dysarthria following subthalamic nucleus deep brain stimulati on for Parkinson's disease. PG - 2434-6 LID - 10.1002/mds.23887 [doi] AB - BACKGROUND: Deep brain stimulation of the subthalamic nucleus (STN-DBS) is an established treatment for patients with Parkinson's disease (PD). Speech impairment is a frequent side effect of the surgery. This study examined t he efficacy of an intensive speech treatment, the Lee Silverman Voice Treatme nt (LSVT) on dysarthria after STN-DBS. METHODS: The LSVT was administered to 10 patients with STN-DBS (surgical group) and 10 patients without (medical gr oup). Patients were assessed before, immediately after, and 6 months following t he speech treatment using sustained phonation, a speech intelligibility scale , and monologue. Vocal loudness, speech intelligibility, and perceptual ratings were the primary outcome measures. RESULTS: Vocal loudness and perceptual score s improved significantly across tasks for the medical group only. Speech intelligibility did not significantly change for either group. Results in the surgical group were variable, with some patients deteriorating. CONCLUSION S: Treatment of dysarthria following STN-DBS needs further investigation beca use of the variable response to LSVT. CI - Copyright (c) 2011 Movement Disorder Society. FAU - Tripoliti, Elina AU - Tripoliti E AD - Sobell Department, Unit of Functional Neurosurgery, UCL, Institute of Neur

ology, FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU LA GR GR GR GR PT PT PT PT DEP PL TA JT JID SB MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTPHSTPHSTPHSTAID PST SO 27. PMIDOWN STATDA Queen Square, London, United Kingdom. e.tripoliti@ion.ucl.ac.uk Strong, Laura Strong L Hickey, Freya Hickey F Foltynie, Tom Foltynie T Zrinzo, Ludvic Zrinzo L Candelario, Joseph Candelario J Hariz, Marwan Hariz M Limousin, Patricia Limousin P eng G-4070/Parkinson's UK/United Kingdom R01 NS040902-10/NS/NINDS NIH HHS/United States R01-NS40902/NS/NINDS NIH HHS/United States Department of Health/United Kingdom Comparative Study Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't 20110927 United States Mov Disord Movement disorders : official journal of the Movement Disorder Society 8610688 IM Deep Brain Stimulation/*adverse effects *Dysarthria/etiology/physiopathology/therapy Humans Middle Aged Parkinson Disease/*therapy Severity of Illness Index Speech Therapy/*methods Subthalamic Nucleus/physiology/surgery Treatment Outcome PMC3223328 NIHMS308492 NLM: NIHMS308492 NLM: PMC3223328 2011/09/29 06:00 2012/04/06 06:00 2011/09/29 06:00 2010/07/14 [received] 2011/06/21 [revised] 2011/06/29 [accepted] 2011/09/27 [aheadofprint] 10.1002/mds.23887 [doi] ppublish Mov Disord. 2011 Nov;26(13):2434-6. doi: 10.1002/mds.23887. Epub 2011 Sep 21952405 NLM MEDLINE 20111123

DCOM- 20120224 LR - 20131016 IS - 1421-9972 (Electronic) IS - 1021-7762 (Linking) VI - 64 IP - 1 DP - 2012 TI - Effect of level of presentation to listeners on scaled speech intelligibil ity of speakers with dysarthria. PG - 26-33 LID - 10.1159/000328642 [doi] AB - OBJECTIVE: This study examined the effect of intensity level of presentati on on scaling of speech intelligibility in speakers with and without dysarthria. PATIENTS AND METHODS: A total of 50 utterances produced by speakers with dysarthria and healthy speakers were played to 60 listeners in four condit ions, which consisted of two different presentation levels ('high' vs. 'low') an d equalization of levels across utterances ('adjusted' vs. 'unadjusted'). Sp eech intelligibility was scaled by using a direct magnitude estimation techniqu e with and without modulus. RESULTS: A significant decrease in speech intelligibi lity was indicated when the stimuli were adjusted to have fixed intensity on th e most intense vocalic nuclei of each word, while no significant change was found between 'high' and 'low' presentation level conditions. CONCLUSION: The fi ndings suggest that an increase in presentation level alone does not result in significant improvement in speech intelligibility ratings. The results are discussed by considering clinical implications in conducting speech therap y with emphasis on intensity variation. FAU - Kim, Yunjung AU - Kim Y AD - Department of Communication Sciences and Disorders, Louisiana State Univer sity, Baton Rouge, LA, USA. FAU - Kuo, Christina AU - Kuo C LA - eng GR - DC00319/DC/NIDCD NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PL - Switzerland TA - Folia Phoniatr Logop JT - Folia phoniatrica et logopaedica : official organ of the International Association of Logopedics and Phoniatrics (IALP) JID - 9422792 SB - IM MH - Adult MH - Dysarthria/etiology/*psychology MH - Humans MH - *Loudness Perception MH - Multiple System Atrophy/complications MH - Observer Variation

MH MH MH MH MH PMC OID EDATMHDACRDTPHSTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI DP TI -

Parkinson Disease/complications *Speech Intelligibility Stroke/complications