neurology case presentation scott m. shorten, md pgy-3
TRANSCRIPT
Neurology Case Presentation
Scott M. Shorten, MDPGY-3
Right-handed man
CC: right facial droop, right arm and leg tingling and weakness
HPI• recurrent drooping of the right face• started 1.5 yrs ago without clear precipitant• multiple times per day and while asleep, no warning, no
trigger• Average 30 minutes (5 min-2 hours), with complete recovery
between• Sometimes associated hand/arm numbness, no other
consistent symptoms• This episode concerning due to ‘stabbing’ mid-frontal
headache with photo/phonophobia, left arm and leg weakness, and lasted over 2 hours. Onset while out in the heat gardening.
• ROS: fatigue, chest discomfort, neck pain
PMHx/SurgHx
• COPD• Hyperlipidemia• Depression• Septic thrombophlebitis, R Cephalic vein
• Appendectomy• Hemorrhoidectomy
Family History
• Mother: Bell’s Palsy, Thyroid disease• Father: Meniere’s Disease• Grandmother: Stroke
Soc Hx
• Married, lives locally • Diesel mechanic• Smokes 1ppd x 30 years• No use of EtOH or Recreational Drugs
Medications
• Verapamil 60mg TID • Carbamazepine 200mg BID• Aspirin 325 qD• Famotidine 10mg qD• Trandolapril 2mg qD• Multivitamin• Simvastatin 40mg qHS• Albuterol PRN
• Allergy: Minocycline
VS: 132/80 36.6 p67 r18
GEN: alert, cooperative, pleasant, NAD.
CV, Pulm, MSK examinations normal
MS: oriented to person/place/time/situation
Speech: slight labial dysarthria. Language normal.
CN: NLF flattened on the right, decreased pinprick Right V1-3*
Motor: Tone and bulk normal, 5/5 throughout
Sensory: decreased pinprick Right UE & LE
Reflexes:
Coordination: normal F-N-F and Heel-shinGait: normal x4, no Romberg
2 22 22 2
1
3
1~ ~
3
?
Workup(occurred over ~1 year)
• Imaging: – MRI of complete neuro-axis: normal– CTA head and neck: normal– Trans-esophageal Echocardiogram: normal– 4-vessel angiogram normal
• Prolonged and Video EEG negative for epileptic event, no slowing, no change on trial of Keppra
• PET: Left lower lobe infiltrate likely pneumonia, no neoplasm
Lumbar Punctures:RBCs WBCs Prot Glu
Presentation 90 20 (88%L) 62 492 days later 2750 15 (51%L) 80 597 days later 140 10 (77%L) 83 601 month later 1 2 70 607 months later1 2 51 6313 months later550 33 (94%L) 76 60
• No growth of bacteria or fungus• Cryptococcal Ab: negative• Oligoclonal bands: negative• IgG index 0.59• ACE: <4• Cytology: negative x4• Extensive workup with ID: unremarkable• Autoimmune/paraneoplastic workup: normal• DRVVT + on 3 months after presentation but normal on
subsequent 6 months later: “possible transient due to viral infection”
• EBV studies: +Capsid IgG +Nuclear ag ab +Early ag ab; - Capsid IgM
??
Mollaret’s Meningitisv.
Pseudomigraine with Temporary Neurologic Symptoms and Lymphocytic Pleocytosis
• started empiric treatment with Acyclovir IV, then Valacyclovir 1000mg daily x 1 year
• Increased verapamil for continued possibility of vasospasm
Mollaret, P. Revue Neurologique. 1944 .Shalabi, M. Clinical Infectious Diseases. 2006.
Mollaret’s Meningitis• Described in 1944• >3 episodes of fever and
meningismus; weeks to years between
• Lasting 2-5 days, wide variation
• Spontaneous resolution• ~50% with neurologic
features
Pierre Mollaret (1898-1987)
• Most commonly due to HSV-2, often with muco-cutaneous lesions found elsewhere
• Diagnosis confirmed with CSF HSV PCR• Valacyclovir prevented genital lesion recurrence
in first year, but no change in meningitis frequencyCanadian Medical Assn. http://www.cmaj.ca/content/174/12/1710.2/F2.expansion.html
Ginsberg L. Pract Neurol 2008;8:348-361 Aurelius E. Clinical Infectious Diseases .2012.
Pseudomigraine with Temporary Neurologic Symptoms and Lymphocytic Pleocytosis
=Migrainous Syndrome with CSF Pleocytosis
=Syndrome of Transient Headache and Neurologic Deficits with CSF Pleocytosis (HaNDL)
HaNDL• First described in 1981• Self-limited, benign condition• Transient neurological deficits
- 15 minutes to 2 hours each, over weeks-months
• Moderate-Severe throbbing headache• Lymphocyte predominant pleocytosis– Avg 199 cells (range 10-760), most >90% Lymph; – avg protein 96, elevated in 96%– Glucose normal– Opening pressure elevated in ~50%
Bartleson, JD. Neurology. 1981Gomez-Aranda, F. Brain. 1997
• Usually in 30s-40s (range 7-52 yrs)• 25-40% had preceding
cough/rhinitis/fatigue/diarrhea• No consistent gender predominance
• Neuroimaging is usually normal– Leptomeningeal enhancement– Hypoperfusion on CT perfusion
• EEG generally shows slowing in the corresponding region
Other Studies
HaNDL Etiology
• Inflammatory/Infectious?– Few reports; Echovirus, HHV-6.
• Migrainous?– SPECT imaging with decreased blood flow at sites
corresponding to neurologic deficit– spreading cortical depression phenomenon
• Infectious, triggering cortical depression?
Castels-van Daele, M. Lancet. 1981.Emond, H. Cephalalgia. 2009.Caminero, AB. Headache. 1997
Diagnosis / Tx• Must first exclude more sinister causes• CSF with >15 cells/mL of lymphocyte
predominance• Episodes of moderate-severe headache
occurring with or shortly following symptoms• Episodes recurring within 3 months
• Symptomatic treatment only, if needed
The International Classification of Headache Disorders: Cephalalgia. 2004
Our Patient
• frequency of attacks 3-4 per day (from up to 20).
• Mostly affecting only his right face• Usually associated with moderate headache• Happy with improvement
Shalabi M, Whitley RJ. Recurrent benign lymphocytic meningitis. Clinical Infect Dis. 2006;43(9):1194.
L Ginsberg, J Kidd. Chronic and Recurrent Meningitis. Pract Neurol 2008;8:348-361.
Aurelius E, Franzen-Röhl E, Glimåker M. Long-term valacyclovir suppressive treatment after herpes simplex virus type 2 meningitis. Clin Infect Dis. 2012;54(9):1304.
Bartleson JD, Swanson JW, Whisnant JP. A migrainous syndrome with cerebrospinal fluid pleocytosis. Neurology. 1981;31(10):1257.
Castels-van Daele M, Standaert L, Boel M, Smeets E, Colaert J, Desmyter J. Basilar migraine and viral meningitis. Lancet. 1981;1(8234):1366.
Caminero AB, Pareja JA, Arpa J, Vivancos F, Palomo F, Coya J. Migrainous syndrome with CSF pleocytosis. SPECT findings. Headache. 1997;37(8):511.
Gómez-Aranda F, Cañadillas F, Martí-MassóJF. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases. Brain. 1997;120 ( Pt 7):1105.