leukoencephalopathy
Table of Contents
leukoencephalopathy
see also:
Introduction
- auto-immune demyelination of the central nervous system mainly affecting white matter especially in children and usually triggered by a viral infection in preceding 1-3 weeks
- can be rapidly progressive over hours sometimes with a poor prognosis
- multiple sclerosis (MS) in contrast is a recurrent flair disease rarely causing decreased mental state and mainly affects adults rather than children
Clinical syndromes
- acute disseminated encephalomyelitis (ADEM)
- aka acute demyelinating encephalomyelitis
- a rare autoimmune disease (8 per 1,000,000 people per year) marked by a sudden, widespread attack of inflammation in the brain and spinal cord
- produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter
- it may be one of the possible clinical causes of anti-MOG associated encephalomyelitis
- unlike MS, ADEM is usually a single event which usually causes decreased conscious state, coma and death (in 5-10%) and occurs usually in children and is marked with rapid fever, although adolescents and adults can get the disease too
- occurs in all ages, most reported cases are in children and adolescents, with the average age around 5 to 8 years old
- adults may have a fulminant cause
- with aggressive Rx, full recovery is seen in 50 to 75% of cases with increase in survival rates up to 70 to 90% with figures including minor residual disability as well
- average time to recover from ADEM flare-ups is one to six months
- cause is often a trigger such as from viral infection (eg. EBV / glandular fever / infectious mononucleosis, measles, COVID-19 coronavirus (2019-nCoV / SARS-CoV-2), etc) or, in extraordinarily rare cases, vaccinations or after organ transplantation
- the only proven vaccine is the Semple form of the rabies vaccine but many other vaccines have been associated with it but at a case study level only
- risk of developing ADEM from an actual measles infection, is about 1 per 1,000 cases whereas risk of ADEM from measles vaccination is 1 per 100,000
- symptoms usually begin 1–3 weeks after infection (or vaccination)
- may have fever, headache, nausea and vomiting, confusion, vision impairment, drowsiness, seizures, coma
- generally have UMN lesion clinical features and may have hemiparesis, paraparesis, cranial nerve palsies and peripheral neuropathy
- neurologic features rapidly progress over the course of hours to days
- Weston-Hurst syndrome
- aka acute hemorrhagic leukoencephalitis
- a hyperacute and frequently fatal form of ADEM - 2% of ADEM cases
- less than 100 cases have been reported in the medical literature as of 2006
- characterized by necrotizing vasculitis of venules and haemorrhage, and oedema
- 70% mortality with death usually within 1 week
- most survivors have neurologic deficits but surprisingly some can survive with little deficit considering the degree of white matter that was damaged
- occasionally associated with ulcerative colitis and Crohn's disease, malaria, sepsis associated with immune complex deposition, methanol poisoning
- cerebrospinal fluid (CSF) shows pleocytosis associated with normal glucose and increased protein
- MRI shows extensive T2-weighted and fluid-attenuated inversion recovery (FLAIR) white matter hyperintensities with areas of haemorrhage, significant oedema, and mass effect
Mx
- supportive care of airways, etc for the unconscious patient as per usual Mx
- high dose IV steroids is usually recommended
- consideration for plasmapheresis, high doses of intravenous immunoglobulin (IVIg), mitoxantrone and cyclophosphamide
leukoencephalopathy.txt · Last modified: 2026/08/27 01:19 by gary1