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Journal of Neurology Neurosurgery and Psychiatry 2002;73:336-339
© 2002 Journal of Neurology Neurosurgery and Psychiatry

SHORT REPORT

Cerebral Whipple's disease with a stroke-like presentation and cerebrovascular pathology

G Peters1, D G Du Plessis2, P R Humphrey1

1 Department of Neurology, Walton Centre for Neurology and Neurosurgery, Liverpool, UK
2 Department of Neuropathology

Correspondence to:
Correspondence to:
Dr P R Humphrey, Department of Neurology, Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool L9 7LJ, UK;
humphr-p{at}wcnn-tr.nwest.nhs.uk


ABSTRACT
Although neurological symptoms are common in Whipple's disease, patients rarely have a purely neurological presentation and involvement restricted to the central nervous system is uncommon. A 39 year old woman presented with a meningoencephalitic illness, which responded to penicillin. Eleven months later she developed recurrent stroke-like episodes. Patchy enhancing meningeal, cortical, and subcortical lesions thought to be vascular in origin developed within nine days of the onset of symptoms. No evidence was found of a cardiovascular source of emboli, vasculitis, or thrombophilic condition. A brain biopsy showed meningoencephalitic features suspicious of Whipple's disease associated with leptomeningeal arterial fibrosis and thrombosis. DNA polymerase chain reaction confirmed Tropheryma whippelii in both blood and brain tissue. The neurological manifestations of cerebral Whipple's disease are varied and very rarely include stroke-like symptoms. The pathogenesis of cerebral infarction in Whipple's disease is not well established but arterial fibrosis and endocarditis complicated by embolisation have been reported. This case emphasises the importance of early brain biopsy in unusual cases of stroke and illustrates the clinical utility of polymerase chain reaction to confirm Whipple's disease.

Keywords: Whipple's disease; Tropheryma whippelii; cerebrovascular disease; stroke; polymerase chain reaction

Abbreviations: ACE, angiotensin converting enzyme; CSF, cerebrospinal fluid; CT, computed tomography; IQ, intelligence quotient; MELAS, mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like syndrome; MERRF, myoclonic epilepsy with ragged red fibres; MR, magnetic resonance; OMM, oculomasticatory myorhythmia; OFSM, oculofacial skeletal myorhythmia; PCR, polymerase chain reaction; WD, Whipple's disease




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