infectious disease clinical case presentation
DESCRIPTION
Infectious Disease Clinical Case Presentation. CC: Acute mental status changes “I feel like I’m going crazy”. History of Present Illness R.S. is a 19 year old white male in the Armed Forces, who was preparing for deployment to Iraq during the week of 9/3. - PowerPoint PPT PresentationTRANSCRIPT
![Page 1: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/1.jpg)
Infectious DiseaseClinical Case Presentation
![Page 2: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/2.jpg)
CC: Acute mental status changes “I feel like I’m going crazy”
![Page 3: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/3.jpg)
History of Present Illness
R.S. is a 19 year old white male in the Armed Forces, who was preparing for deployment to Iraq during the week of 9/3.
The patient’s family visited him over the weekend (9/1-9/2) and he was in a normal state of health aside from complaints of a headache.
![Page 4: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/4.jpg)
On Monday, 9/3, his father called him around 1300 and was surprised to find him still in bed. His son sounded unusually sleepy.
That evening the patient told his mother that he with felt like he was “going crazy.”
On 9/4 he did not show up for work.
![Page 5: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/5.jpg)
On 9/4 he did not show up for work.
He was found on his bed nude, mumbling incomprehensible words.
He was taken to AF base facility and then to Wayne Memorial.
Upon admission, he could state his first name and the year.
He began showing signs of frontal disinhibition and rapidly deteriorated.
![Page 6: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/6.jpg)
He underwent lumbar puncture and was placed on ceftriaxone, vancomycin, and acyclovir.
He was intubated for airway protection and transferred to MICU at UNC.
Upon arrival patient was minimally responsive to noxious stimuli.
![Page 7: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/7.jpg)
Past Medical History: Previously healthy
SOCIAL HX: Active duty stationed at a nearby airforce base. Deployment week of 9/3/07. Per coworkers, patient does not drink, smoke, or do illicit drugs.
Travel: Patient trained in Texas November-April and then moved to North Carolina. He visited family in NY state in July.
FAMILY HX: No hx of early CAD
HOME MEDICATIONS: mefloquine
ALLERGIES: NKDA
![Page 8: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/8.jpg)
• Review of Systems• Other than HPI fairly unobtainable. • Mother had noticed a rash on his feet
bilaterally on Tuesday, 9/5 at Wayne Memorial. She thought it might have been due to the boots he had been wearing.
![Page 9: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/9.jpg)
Vitals Tmax = 39.4 on admission BP 115/59 RR 20
Physical Exam:
General: Intubated, no response to voice.
Lymphadenopathy: 1 mm left axillary node
Skin: Two to three 1 mm areas with blanching papules bilaterally on the feet. Similar papules were on the dorsum of PIP on left hand and DIP of ring finger on left hand.
![Page 10: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/10.jpg)
Physical Exam: Neurological
Comatose, no response to voice
Visual fields show no reaction to threat bilaterally, PERRLA
Normal bulk and tone, bilateral upper extremity extensor posturing with nail pressure
Slight withdrawal on left lower extremity with nail pressure, slight movement of right quadricep with right lower extremity naill pressure
Reflexes symmetric and 3+ bilaterally at bicep, tricep, bracheoradialis, patellar, ankle.
![Page 11: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/11.jpg)
ADMISSION DIAGNOSTIC STUDIES: (WM)
CBC:
WBC 12.7
Gran 10.7 (84.3%)
Lymph 1.5 (11.7%)
Mono 0.5 (3.8%)
Eos 0.0
Baso 0.0
RBC 4.74
Hemoglobin 13.6
Hematocrit 41
Platelets 215
Chem panel:
Na 138
K 4.2
Cl 102
BUN 27
Creatinine 11
Anion gap 9
LFTs normal range UA: trace protein, ketones 15, rare WBCs
EKG: NSR, biatrial enlargement
![Page 12: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/12.jpg)
ADMISSION DIAGNOSTIC STUDIES
Toxicology Screen Negative:
Amphetamines
Barbiturates
Benzodiazepines
Cocaine
Opiates
Phencycidine
Cannaboids
TCAs
Methamphetamine
Methadone
Toxicology screen positive:
Acetaminophen
![Page 13: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/13.jpg)
ADMISSION DIAGNOSTIC STUDIES
LP: opening pressure 36
Appearance: colorless clear
RBCs 3,
WBCs 135
28% neutrophils 59% lymphocytes, 13% monocytes,
Glucose 65, Protein 91.
CSF Gram stain: No organisms, few WBCs
![Page 14: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/14.jpg)
ADMISSION DIAGNOSTIC STUDIES
CT with and without contrast: showed “no acute intracranial process and no enhancing lesions.”
An MRI was performed at Wayne Memorial prior to transfer.
MRI also performed at UNC on evening of arrival to MICU.
![Page 15: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/15.jpg)
MRI BRAIN 9/5/07: T2 Images
![Page 16: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/16.jpg)
![Page 17: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/17.jpg)
![Page 18: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/18.jpg)
![Page 19: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/19.jpg)
![Page 20: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/20.jpg)
![Page 21: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/21.jpg)
![Page 22: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/22.jpg)
![Page 23: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/23.jpg)
![Page 24: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/24.jpg)
![Page 25: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/25.jpg)
![Page 26: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/26.jpg)
![Page 27: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/27.jpg)
![Page 28: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/28.jpg)
![Page 29: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/29.jpg)
MRI BRAIN: FLAIR Images
![Page 30: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/30.jpg)
![Page 31: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/31.jpg)
![Page 32: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/32.jpg)
![Page 33: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/33.jpg)
![Page 34: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/34.jpg)
![Page 35: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/35.jpg)
![Page 36: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/36.jpg)
![Page 37: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/37.jpg)
![Page 38: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/38.jpg)
![Page 39: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/39.jpg)
![Page 40: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/40.jpg)
![Page 41: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/41.jpg)
![Page 42: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/42.jpg)
![Page 43: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/43.jpg)
FINDINGS There are large areas of abnormal T2 and FLAIR signal abnormalities involving the subcortical and deep white matter in the bilateral frontal, parietal, and occipital lobes.
There is abnormal signal involving the the corpus callosum and periventricular white matter.
There is abnormal increased T2 and FLAIR signal involving the medial portions of the temporal lobes and right thalamus.
There is similar abnormal signal involving the posterior pons.
There is a somewhat linear area of restricted diffusion in the left frontal region just superomedial to the sylvian fissure. This correlates with an area of FLAIR and T2 signal abnormality.
There is abnormal FLAIR signal in the subarachnoid spaces bilaterally superiorly. This is nonspecific but can be seen with proteinaceous fluid or subarachnoid hemorrhage but can also be related to ventilation.
IMPRESSION
1.Multiple areas of abnormal signal involving predominantly white matter but also areas of gray matter.
2. Nonspecific increased FLAIR signal in the subarachnoid space as described above.
![Page 44: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/44.jpg)
DISCUSSION
![Page 45: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/45.jpg)
Additional History: Vaccine History
•On 8/18 pt received anthrax vaccine #1, as well as typhoid vaccine IM
•On 8/23 patient received a smallpox vaccination left deltoid.
•On 8/30 he received anthrax vaccine #2
![Page 46: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/46.jpg)
Course:•Upon arrival to UNC his smallpox vaccination site was examined by Dr. Weber and found to be a “8 mm well scabbed over black eschar on left upper arm.”
•He had no evidence on exam for satellite lesions.
•Patient was placed on contact precautions.
•ICU team added Doxycycline.
•ID and Neurology were consulted.
![Page 47: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/47.jpg)
LABS
HIV negative RPR NR Crypto ag serum neg B12 normal
TSH normal
---------------------------------------------------------------------------------------------------
WNV (CSF) VZV PCR (CSF) HSV PCR (CSF) Lyme titer (CSF)
Crypto Ag CSF Fungal and AFB stain and culture (CSF)
VDRL (CSF)
![Page 48: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/48.jpg)
Course:•We recommended addition of high dose ampicillin to cover Listeria in addition to continuing vancomycin, ceftriaxone, acyclovir and doxycycline.
•Asked MICU to check RMSF titers, arbovirus serologies.
•We were most concerned for a post vaccinia encephalitis (PVE).
•Neuro-radiology and Neurology: Imaging, clinical picture c/w Acute Disseminated Encephalomyelitis (ADEM).
•Neurology recommended high dose steroids and IVIG.
![Page 49: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/49.jpg)
Consultation with the CDC and DOD on 9/5/07:
A second LP at WM had been done on 9/4 with CSF and serum sent to CDC labs.
Poxvirology Lab:
PCR negative CSF and Blood for poxvirus nucleic acid
Serum and CSF IgG negative for poxvirus
Serum and CSF IgM pending
CDC strongly endorsed adding IVIG to the high dose steroids.
![Page 50: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/50.jpg)
CDC Conference Call
CDC also recommended several additional tests:
• Pre-IVIG Serum sent to CDC for Poxvirus antibody testing.
• highly sensitive CRP
• complement levels and circulating immune complexes
•EBV, CMV DNA PCR, serologies in blood
•Chlamydia antibodies
•Streptoccoccal antibodies
Conference calls with the CDC were continued to follow the course of the post vaccinia complication: post vaccinial encephalitis.
![Page 51: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/51.jpg)
CDC Conference Call:
Later that evening the serum and CSF IgM returned positive.
Despite lack of evidence for disseminated vaccinia, decided patient might benefit from vaccinia immunoglobulin and CDC shipped VIG overnight to RDU.
VIG started on Friday afternoon, IVIG resumed afterwards until pt completed 2g/kg over 4 days.
High dose steroids continued.
![Page 52: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/52.jpg)
Course
•Vancomycin, ceftriaxone, doxcycline dc’d after >48 hrs negative cultures, low susp for RMSF.
•Ampicillin continued until final CSF cultures negative 9/10.
•Acyclovir continued until CSF HSV, VZV PCR negative 9/11
![Page 53: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/53.jpg)
Course
•West Nile CSF negative
•Lyme antibody CSF negative
•RMSF serum IFA 1:80
•CDC labs: CSF negative for Adenovirus, Enterovirus, HSV, VZV
•CDC testing of serum: now positive for IgG on pre IVIG, pre VIG, post VIG serum
![Page 54: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/54.jpg)
Vaccinia virus is a live DNA virus used as the vaccine against smallpox, which is caused by the Variola virus.
Genus: Orthopoxvirus.
Day 3-5 Papule
Day 5-8 Vesicular
Day 8-10 Pustular
Day 14-21 Scab separation
![Page 55: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/55.jpg)
Adverse events after smallpox vaccination recommended for report:Superinfection of the vaccination site or regional lymph nodes
Inadvertent inoculation
Contact transmission
Ocular vaccinia
Generalized vaccinia
Eczema vaccinatum
Progressive vaccinia
Erythema multiforme major or SJS
Fetal vaccinia
Postvaccinial CNS diseasePostvaccinial CNS disease
Myo/pericarditis
Dilated cardiomyopathy
![Page 56: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/56.jpg)
Adverse Events
• Inadvertent Inoculation– Results in a normal vaccinial lesion in an
inappropriate site (most common complication in 1968 study).
![Page 57: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/57.jpg)
Adverse Events
• Generalized Vaccinia– Generalized vaccinia is the result of the
systemic spread of virus from the vaccination site. Despite the appearance of the lesions, it is usually a benign complication of primary vaccination that is self-limited except in some individuals with underlying immunosuppression (medications or illnesses).
![Page 58: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/58.jpg)
Adverse Events
• Eczema Vaccinatum– A local or disseminated vaccinia that occurs
in patients with a hx of eczema or other types of atopic dermatitis
• Erythema Multiforme– Pathogenesis thought to be allergic, toxic, or
both.
![Page 59: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/59.jpg)
Adverse Events
•Progressive Vaccinia (Vaccinia Necrosum)– Universally fatal prior to VIG– Occurs in immunodeficient vaccinees– Progressive destruction of local areas of
skin, subcutaneous tissue, and metastatic lesions can lead to death
![Page 60: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/60.jpg)
Adverse Events
• Myocarditis and Pericarditis– Effects range from asx T wave changes to
fatal myocarditis.– During 2003 civilian first responders
vaccination program, 6 out of 10,000 vaccinees developed myocarditis.
![Page 61: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/61.jpg)
Post-Vaccinial Encephalitis
•Neurological illness is a rare but severe Vaccine Adverse Event (VAER)
•Post-vaccine Encephalitis (PVE)
•Historically occurred with greater frequency in first time vaccinees.
![Page 62: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/62.jpg)
Case Definition of PVE for use in Smallpox VAER(Sejvar et al JAMA 2005)
•Confirmed PVE: •acute cerebral +/- menningeal inflammation or demyelination on histopathology
•Probable PVE:
•Encephalopathy (AMS, personality change) >24 hrs
•AND
•Additional features suggestive of cerebral inflammation including 2 or more of following:
•Fever (>38) or Hypothermia (<35)•Meningismus•Pleiocytosis•Presence of focal neurologic defect•EEG c/w encephalitis•Neuroimaging (MRI) c/w inflammation or demyelination•Seizures
•AND
•No alternative etiology
•Suspected PVE: same as Probable except that only one criteria for cerebral inflammation or demyelination.
![Page 63: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/63.jpg)
Post-Vaccinial Encephalitis
•Clinicohistopathologic data from the 1920s and 1960s identified 2 clinicopathological forms of PVE:
•Microglial encephalitis
•Post-vaccinial encephalopathy
![Page 64: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/64.jpg)
PVE: Microglial encephalitis:
–More frequent in >2 years of age
–10-20 days after vaccination
–Fever, vomiting, headache malaise followed by decreased consciousness, seizures, coma
–Widespread demyelination of subcortical white matter (prob corresponds to ADEM)
ie MRI etc were not available in 1920s, 1960s.
![Page 65: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/65.jpg)
PVE: Post-vaccinial encephalopathy
– More frequent in <2 years of age– 6-12 days after vaccination– Fulminant seizures and hemiplegia, elevated ICP.– Diffuse cerebral edema and perivascular
hemorrhages– At times vaccinial viremia and even vaccinia virus
isolation/detection from brain or CSF.– A neuroinvasive form of vaccinia virus?
![Page 66: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/66.jpg)
European Countries (1964)
Incidence %
Britain 1.5
Finland 3.1
Sweden 3.5
Switzerland 5.0
Belgium 7.0
Holland 13.0
Germany 11.0
Austria 30.0
The overall incidence in the U.S. was 2.9/million vaccinees in 1968. The case fatality rate in the U.S. was 25% and 30-50% in Europe (1959-1966). In 2001, the CDC reported the rate as 1 case per 300,000 vaccinees.
POST VACCINIAL ENCEPHALITIS
![Page 67: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/67.jpg)
Acute Demyelinating Encephalomyelitis (ADEM)
• ADEM is an immune mediated inflammatory disorder of the CNS, primarily of the white matter, that is typically precipitated by viral infection or vaccination
![Page 68: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/68.jpg)
ADEM
•Diagnosis of exclusion
•Differential: Infection, MS, Transverse Myelitis
•Based on clinical and radiologic features (MRI critical)
•Usually monophasic , recurrent ADEM has been reported
![Page 69: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/69.jpg)
Clinical FeaturesADEM
• Rapid onset encephalopathy• Prodrome with fever, malaise, headache,
nausea, vomiting• Meningeal signs and drowsiness• Rapidly progressive, developing over hours to
maximum deficits within days • Neurologic signs include acute hemiplegia,
ataxia, cranial nerve palsies, seizures, impairment of speech, mental status changes
![Page 70: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/70.jpg)
MRI FeaturesADEM
• Patchy, poorly marginated areas of increased signal intensity; large, asymmetric, multiple
• Four patterns:– ADEM with less than 5 mm lesions– Large, confluent lesions with edema and mass
effect– ADEM with additional symmetric bithalamic
involvement– Acute hemorrhagic encephalomyelitis (worst
prognosis)
![Page 71: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/71.jpg)
Epidemiology of ADEM
• More common in pediatric patients• Recent study of persons less than 20 years
with ADEM showed 5% had a vaccination within 1 month, 93% had signs of infection in preceding 21 days
• Post-vaccinial encephalitis usually occur 7-14 days after vaccination
• Incidence varies by country
![Page 72: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/72.jpg)
Pathophysiology of ADEM
• Pathogenesis is not well understood• Immune pathogenesis supported by
time course between vaccine and encephalitis
• Similarity in the neuropathology of ADEM with animal models of experimental allergic-autoimmune encephalitis (EAE)
![Page 73: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/73.jpg)
Pathophysiology of ADEM
• EAE is an autoimmune disease mediated by T cells directed at myelin antigens
• Postulated that phosphorylation of myelin basic protein, by vaccinia’s viral kinase, may change the immunogenicity of myelin basic protein
• Viral epitopes may resemble myelin reactive T cell clones through molecular mimicry
![Page 74: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/74.jpg)
Treatment of ADEM
• No standard therapy• Based on case reports and small series• Most therapies use a form of
immunosuppressant therapy– Steroids– IV immunoglobulin – Plasmapheresis
![Page 75: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/75.jpg)
Treatment of ADEM
•IVIG– Interaction with Fc receptors on effector cells – Anti-idiotypic antibodies against circulating
antibodies– May alter the number of T cells and subsets– Promote clearance of immune deposits– May contain neutralizing antibodies– May increase clearance of pathogenic IgG– May neutralize the inflammatory actions of
complement
![Page 76: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/76.jpg)
Treatment of ADEM
•High dose steroids–Gastric perforation, hyperglycemia, hypokalemia, hypertension, facial flushing
•Plasmapheresis–Hypotension, bleeding, allergic rxn, immunosuppresion
•* Vaccinia immunoglobulin
![Page 77: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/77.jpg)
VIG and ADEM due to PVE?
•Vaccinia Immunoglobulin (VIG) not thought to be useful because PVE thought to be immune mediated, and not due to vaccinial infection.
•Nanning et al, 1962: Randomized trial of prophylactic VIG in >106,000 Dutch troops vaccinated with smallpox vaccine reduced incidence of PVE from 13 to 3
![Page 78: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/78.jpg)
PrognosisCase reports
•recovery•Mild to severe impairment•Fatalities•ADEM with 15 cases had a 50% recovery rate
Pooled summary of case fatality rates (CFR)–For every million primary vaccinations
•60 cases accidental infection•40 cases of generalized vaccinia•13 cases eczema vaccinatum•3 cases of post-vaccinial encephalitis (CFR 28.9%)•1 case of vaccinia necrosum (CFR 15.4%)
![Page 79: Infectious Disease Clinical Case Presentation](https://reader036.vdocument.in/reader036/viewer/2022062500/56815949550346895dc68446/html5/thumbnails/79.jpg)
Current clinical course
• Patient has no spontaneous extremity movements
• Patient has opened his eyes, and is moving his eyes in response to voice and movement
• Continue tapering dose of steroids and watch for improvement