Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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July 18, 2019
Acute parenchymal neuro-Behçet’s disease
- Background
- Behçet’s disease is a multisystem inflammatory disorder of undetermined etiology; neurological involvement occurs in 5.3–14.3% of patients (neuroBehçet, NBD).
- Central nervous system disease can be divided into parenchymal (brainstem, cerebral, spinal cord, multifocal) and non-parenchymal (cerebral venous and arterial vessel disease, venous thrombosis, intracranial hypertension syndrome, and acute meningeal syndrome).
- Clinical Presentation
- Typically the initial manifestation is a subacute meningeal syndrome, followed either by symptoms and signs of brainstem involvement (ophthalmoparesis, cranial neuropathy, cerebellar syndrome), spinal cord, or cerebral hemispheric involvement.
- Less commonly, a progressive subcortical dementia may develop.
- Usually signs of exacerbation of the systemic disease are also present (orogenital ulcers, skin lesions, uveitis).
- Key Diagnostic Features
- In acute or subacute parenchymal NBD, typical hyperintense lesions in T2-weighted (T2) or FLAIR images and hypo to isointense on T1-weighted (T1W) images involving the brainstem, particularly the pons.
- The lesions may extend upwards to involve the midbrain, basal ganglia, thalami and the diencephalon.
- Patchy enhancement on T1W contrast sequences is common. Optic tract involvement, as in our case, has been rarely described.
- Lesions may decrease in size after treatment and even disappear (an almost complete regression was seen after brief corticotherapy in the present case).
- Differential Diagnosis
- Other inflammatory diseases:
- Neurosarcoidosis – Cranial nerve, pituitary and hypothalamic involvement, patchy periventricular T2W white matter hyperintensities (WMH), focal or generalized leptomeningeal involvement seen in T1W post-gadolinium.
- Neuromyelitis Optica – Optic nerve involvement, lesions of the periaqueductal grey matter, hypothalamus/medial thalamus and the dorsal pons/medulla (area postrema). Less often, there can be patchy periventricular T2W WMH or large hemispheric lesions.
- Multiple Sclerosis – Multiple T2W WMH, rarely symmetric, typically ovoid and with perivenular location (Dawson’s fingers).
- Infectious diseases:
- Enterovirus rhomboencephalitis – The pontine tegmentum is the most common site of brainstem involvement. Usually there is no supratentorial involvement.
- Japanese and West-Nile virus encephalitis – T2W hyperintensity and restricted diffusion of basal ganglia and thalami.
- Metabolic disorders:
- Wernicke Encephalopathy – Variable T2W hyperintensity of the periaqueductal gray matter, dorsomedial thalami and mamillary bodies.
- Leigh disease – Variable and usually symmetrical lesions in corpus striatum, brainstem and dentate nuclei.
- Metronidazole Intoxication – Symmetrical high T2 signal intensity in the cerebellum, mostly of the dentate nuclei. Less often of corpus callosum or midbrain.
- Other inflammatory diseases:
- Treatment
- High dose corticosteroids during acute relapses.
- Long-term immunosuppressors, including azathioprine, mycophenolate mofetil, metotrexate or biological agents, particularly TNF-alfa inhibitors are important disease modifiers to prevent relapses.
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