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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April 15, 2021
Neurobrucellosis
- Background:
- Brucellosis is a multisystem zoonotic disease usually transmitted to humans via ingestion of uncooked meat or unpasteurized dairy products.
- Neurologic disease occurs in 5–10% of cases and may affect peripheral or central nervous systems.
- Clinical Presentation:
- Clinical findings are diverse and nonspecific. Fever, headache, arthralgia, and myalgia are common general symptoms.
- Sensorineural hearing loss is common. Other neurologic manifestations include: behavioral changes, confusion, blurred vision, weakness, and cranial nerves neuropathy.
- Key Diagnostic Features:
- Imaging abnormalities can be categorized into 4 patterns: normal, white matter changes, inflammation, and vascular changes.
- White matter changes can be focal, diffuse peripheral, or periventricular.
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Inflammatory pattern manifests as enhancement of the meninges or lumbar nerve roots as well as enhancing suprasellar granuloma.
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Cerebrovascular involvement leads to lacunar infarcts, small hemorrhages, and venous thrombosis. Embolic disease and mycotic aneurysms may result from Brucella endocarditis.
- Diagnosis is confirmed by isolation of Brucella species or anti-Brucella antibodies from CSF.
- Differential Diagnoses (for white matter changes pattern):
- Multiple sclerosis: Plaques preferentially affect the callososeptal interface. Active lesions show enhancement (solid or peripheral, both incomplete and complete).
- Monogenic small vessel disease (SVD):
- CADASIL: In addition to anterior temporal lobe and external capsule involvement, multiple lacunar infarcts in basal ganglia, thalami, and brainstem are common. Microhemorrhages can also be found.
- COL4A: Hemorrhages, areas of cavitation, and intracranial aneurysms
- PADMAL: A variant of COL4A shows pronounced atrophy of the pons.
- There are other known diseases that show usually nonspecific WM abnormalities which are more pronounced than expected for the patient's age (HTRA-1, CARASIL, CARASAL). The key may be in the family history and confirmed with genetic testing. Other diseases are beyond the scope of this case (Fabry, RVCL-S, hereditary AA).
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Angiitis of the CNS: Can be primary, or more commonly, secondary. Subcortical white matter and basal ganglia involvement is characteristic. Patchy enhancement is commonly seen. Acute ischemic lesions demonstrate diffusion restriction. VWI may show medium vessel contrast enhancement or leptomeningeal enhancement when it is only small vessel disease.
- Treatment:
- Multiple antimicrobial drugs including rifampicin, doxycycline, and co-trimoxazole
- Sensorineural hearing loss, white matter changes, and ischemic changes were observed to persist after treatment and normalization of CSF.
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