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Published byHilary Clarissa McDowell Modified over 9 years ago
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30 y.o. ICU assistant 2 Weeks prior to admission--fever, chills, myalgias along with bad headaches St. Paul ER--diagnosed with UTI and started on Levaquin 1 Week prior to admission seen in PHD ER and diagnosed with flu On day of admission (9/6/03), found on floor unable to get up with shaking, and ?seizures
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Exam Unresponsive. Eyes tended to deviate downward and to the right. Corneals intact. Followed no commands. On a respirator Marked increased tone in the extremities, with flexion at the elbows and extension of the legs DTR’s increased. Plantars flexor. LP done: 34 WBC’s, mostly lymphs, protein 179, glucose 34 Fungal antigens, cultures, PCR for herpes, etc. all negative in CSF
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Initially started on Rocephin; had also been started on acyclovir
After MRI, started on 1gm solu medrol per day No change in neuro exam and brain biopsy done on 9/10/03 Biopsy initially read as meningitis and possible encephalitis, and even a diagnosis of vasculitis entertained. No improvement and antibiotics discontinued and solu medrol tapered. Then because of worsening changes on MRI, IV solu medrol increased again and given a course of IVIG around 10 days after admission Never any improvement and after a few weeks, she remained unresponsive with posturing of the extremities and marked increased tone
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Brain Biopsy Findings Light microscopy:
perivascular chronic inflammation Predominant white matter findings of macrophage infiltrate consistent with demyelination chronic inflammation in leptomeninges no granulomas, vasculitis or viral cytopathic changes stains for bacteria, fungi, AFB, HSV, varicella, CMV, measles, adenovirus, EBV and bacilli associated with Whipple’s Disease negative Electron microscopy: evidence of myelin breakdown no viral organisms identified
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Differential Diagnosis
Acute disseminated encephalomyelitis viral encephalitis subacute sclerosing panencephalitis PML (progressive multifocal leukoencephalopathy) Whipple’s Disease multiple sclerosis metabolic leukodystrophy
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Viral Encephalitis neuronophagia with microglial nodules
perivascular lymphocytes nonsuppurative leptomeningitis intranuclear inclusions predominantly gray matter parenchymal necrosis with macrophage infiltrate
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Subacute Sclerosing Panencephalitis
chronic progressive encephalitis past exposure to measles virus by several years patchy demyelination, IN inclusions, gliosis
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Progressive Multifocal Leukoencephalopathy
caused by JC, polyoma virus immunocompromised patients demyelination, IN inclusions in oligodendrocytes
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Whipple’s Disease chronic multisystem disorder
gram positive bacillus Tropheryma heavy macrophage infiltrate in organs with PAS+ rods (small bowel, CNS)
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Multiple Sclerosis chronic progressive demyelinating disorder
plaques of different ages usually associated with persistent oligoclonal bands of immunoglobulins
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Metabolic Leukodystrophy
inherited metabolic disorders affecting white matter usually infants, children demyelination and macrophage accumulation
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Acute Disseminated Encephalomyelitis (ADEM)
Immune mediated inflammatory demyelinating encephalomyelitis Usually preceded days to weeks before by an antecedent event such as viral illness or inoculation Acute onset of fever, headache, and rapidly followed by meningeal signs, altered consciousness, and focal central neurological signs Young adults and children most commonly affected
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Acute Necrotizing Hemorrhagic Encephalopathy (ANHE)
Similar to ADEM Much more explosive in onset Characterized by hemorrhage which can be extensive Usually fatal
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Differential Diagnosis
Viral encephalitis as well as other infectious etiologies The history of a period of relative well-being following a febrile illness suggests ADEM In this case, the distribution of the abnormalities predominantly in the white matter would be unusual for viral encephalitis Negative serologies and cultures would favor ADEM Even biopsy may not always be conclusive Vasculitis Usually see multiple areas of infarction evident on diffusion weighted images and FLAIR Diagnosis suggested by Agram and confirmed by biopsy
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Relationship to Multiple Sclerosis
ADEM Multiple Sclerosis Inciting antigenic stimulus Monophasic illness though relapses in up to 20% Alteration of consciousness common Oligoclonal bands absent 14/40 adult patients developed MS 0/31, 10/25 children developed MS No causal relationship to infection or inoculation Polyphasic with relapses and remittances or slow progression Alteration of consciousness rare and coma unheard of Oligoclonal bands present Fever/meningismus rare
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Optic Neuritis Occurs in 90% of multiple sclerosis patients
13-85% progress to multiple sclerosis. Predictors: More than 4 lesions on MRI of brain (85%) Presence of oligoclonal bands RR 5.2 HLA-DR2 RR 3.2 Unilateral optic neuritis more likely to progress to MS
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Transverse Myelitis 1/3 of idiopathic give history of URI or flu-like illness Less than 10% progress to multiple sclerosis Devic’s disease (neuromyelitis optica)
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Other Demyelinating Disease
Leukodystrophies Progressive multifocal leukoencephalopathy Immunocompromised individuals JC virus or polyoma SV40 Heavy involvment of U fibers evident on MRI
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Viral Infections ADEM in 1/1000 measles infections Rubella
Mortality 25%; 25-40% of survivors permanent neurological impairment Myoclonus common Must be distinguished from SSPE Rubella 1/500 Varicella-zoster 1/10,000 An acute cerebellar syndrome common Mumps Hemiplegia common Others: herpes simplex, influenza, HIV, EB virus
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Bacterial Infections Most commonly mycoplasma Others: Streptococcus
chlamydia legionella campylobcter
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Vaccines Rabies--common in the past(1/400), almost non-existent now with human diploid cell vaccine DTP Smallpox 1/4,000 Measles 1-2/million Japanese B encephalitis Polio Hepatitis B Influenza
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Treatment Methylprednisolone IVIG Plasmapharesis
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Prognosis 40 adult patients 31 Children 14 developed MS
Of remaining 26: 2 died 9 had minor residual deficits and 3 modt. Deficits 12 were completely recovered 31 Children 81% recovered completely In the remaining 5 patients, only mild neurological sequelae 4 had relapses
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