Faculty Bibliography

A 39-year-old intravenous drug user presented with dysarthria and a syndrome of the left cerebellar hemisphere. While in hospital, he developed progressive brainstem findings. Repeated CT scans revealed a lucency in the white matter of the left cerebellar hemisphere. Brainstem auditory and short-latency somatosensory evoked potentials provided evidence of brainstem dysfunction without corresponding lesions on CT. Biopsy of the cerebellum established the diagnosis of progressive multifocal leukoencephalopathy (PML) and the acquired immunodeficiency syndrome (AIDS). Postmortem examination revealed brainstem lesions appropriate to the evoked potential findings and a radiographically inapparent lesion in the right internal capsule. Based on this case and a review of the literature we conclude that: (1) PML occurs with significant prevalence in AIDS patients and may involve the posterior fossa; (2) the diagnosis of posterior fossa PML is suggested by certain clinical and radiographic criteria and may be confirmed by brain biopsy; (3) evoked potentials may be abnormal in PML and can reveal functional abnormalities of white matter without apparent CT abnormalities.

Fatigue is a frequent symptom in multiple sclerosis (MS) that can interfere with a patient's daily functioning. The cause of MS fatigue, its clinical characteristics, and its relationship to other symptoms remain poorly understood. Structured interviews were conducted with 32 patients with MS and 33 normal healthy adults. Fatigue proved to be both more frequent and more severe among the patients with MS. Multiple sclerosis fatigue was unrelated to either depression or global impairment. Multiple sclerosis fatigue appears to be a distinct clinical entity, often disabling, that can be distinguished from normal fatigue, affective disturbance, and neurologic impairment.

Nonpsychiatric and nonobstetrical principal diagnoses of 527 prison inmates discharged in 1981 from one referral hospital were reviewed. Male prisoners had the following discharge diagnoses more frequently than an age-matched and sex-matched sample of the general population: lymphadenopathy, viral hepatitis, foreign body insertion into the gastrointestinal tract, dental caries, and pulmonary tuberculosis. Some differences may be due to lifestyles preceding incarceration, others may result from conditions of the prison environment.

To characterize the neurological complications of cancer patients admitted to a community hospital, the charts of all cancer patients evaluated by a neurologist during a single year were reviewed. Nine percent (N = 93) of cancer patients received neurological consultation compared to 3.6% of other patients. The neurologic problem preceded the diagnosis of cancer in 11% of patients. Complications were most common with known metastases. Neurologists frequently discovered signs not noted by the referring physician: 52 patients were paretic, with weakness reported in only 31; cranial nerve complaints were described in 3, but found in 20; sensory abnormalities were noted in 8, but found in 26. A change in mental status was confirmed in 33% and ataxia in 10%. After consultation, distant metastasis was diagnosed in 40% of patients, direct extension in 8%, metabolic encephalopathy in 14% and remote effects of cancer and side effects of cancer therapy in 4% each. Other patients had unrelated diagnoses. In most cases, the neurologic consultation let to a change in treatment, with radiotherapy directed to a symptomatic tumor mass the most common beneficial outcome.

Between April 1982 and March 1984 7 pathologically confirmed cases of progressive multifocal leukoencephalopathy (PML) were diagnosed at our institution. Only 1 case had been seen in the preceding twenty years. Four patients had acquired immunodeficiency syndrome (AIDS). The others had chronic lymphocytic leukemia, Hodgkin's lymphoma, and systemic lupus erythematosus. All patients presented with progressive neurological deficits. In most, the initial computed tomographic (CT) scan was disproportionately less abnormal than the clinical findings. In 5 patients the first CT scan revealed hypodensities of the cerebral white matter which lacked mass effect and did not enhance with contrast agent. The lesions were observed to enlarge progressively on CT scans but often lagged behind the rate of clinical evolution. Although 3 patients were treated with cytosine arabinoside, none improved. PML had similar clinical, radiographic, and pathological features in the AIDS and non-AIDs patients. Of 79 AIDS patients cared for at our institution between December 1979 and December 1983, 3.8% had PML. PML should be suspected in AIDS patients in the presence of the characteristic CT features, especially when CT-clinical dissociation occurs.