Brain Pathology Case of the Month - July 1999

Contributed by Juan M. Bilbao, MD.
    University of Toronto, Department of Pathology, St. Michael's Hospital, Toronto, Ontario, Canada, M5B 1W8
Published on line in July 1999


Several weeks after a tooth extraction complicated by a dental abscess that required ampicillin and metronidazole, a 66 year old retired mechanic, born in Barbados, was admitted to hospital with a one week history of urinary retention, change in personality and headache. Medical history was notable for hypertension, duodenal ulcer, a positive TB skin test and microscopic haematuria. Initial examination revealed a disoriented, ataxic and dysarthric man. He exhibited generalized lynphoadenopathy, and an exfoliative macular rash. The cranial nerves were normal and there was weakness of the proximal musculature with absent reflexes in legs and a Babinski sign on the left. Sensory examination revealed hyperesthesia and allodynia below the knees. Following admission the patient manifested polyarthritis, became drowsy and developed respiratory failure. Laboratory results included normal biochemistry except for elevated creatinine (160 nml/L), elevated CPK (1.5x the upper limit of normal). Hemoglobin was 71g/l with positive direct Coombs test. WBC count was 3.2 x109/L, and platelets were normal. ESR was 120 mm/hr. CSF examination disclosed a normal cell count and glucose, with markedly elevated protein of 2 g/L .Neuroimaging showed slight cerebral atrophy and no focal lesions. EMG and NCS disclosed a diffuse axonal polyneuropathy. The following tests and procedures produced normal results: Chest X-ray, urine porphobilinogen, blood cultures, heterophile antibodies, HIV, HTLVI/II, bone marrow and lymph node biopsies, rheumatoid factor, ANCA and imaging of many organs.



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