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Background: Inhalation of heated heroin vapor ("chasing the dragon"), which is gaining popularity among drug users seeking to avoid the risks of parenteral drug administration, can produce progressive spongiform leukoencephalopathy., Methods: We studied the clinical phenotype and course, MRI, MRS, and brain pathology in the first American patients described with this syndrome., Results: Two of the three heroin users studied inhaled heroin pyrolysate together daily over the course of 2 weeks. They developed ataxia, dysmetria, and dysarthria. Patient 1 progressed to an akinetic mute state with decorticate posture and subsequent spastic quadriparesis. Patient 2 developed a mild spastic quadriparesis and gait freezing. Patient 3 was asymptomatic following less heroin exposure. Brain MRI showed diffuse, symmetrical white matter hyperintensities in the cerebellum, posterior cerebrum, posterior limbs of the internal capsule, splenium of the corpus callosum, medial lemniscus, and lateral brainstem. MRS showed elevated lactate. Brain biopsy (Patient 1) showed white matter spongiform degeneration with relative sparing of U-fibers; electron microscopy revealed intramyelinic vacuolation with splitting of intraperiod lines. Progressive deterioration occurred in Patients 1 and 2 over 4 weeks. Both were treated with antioxidants including oral coenzyme Q, and clinical improvement occurred. Patient 1 recovered nearly completely over 24 months. Patient 2 improved, but developed a delayed-onset cerebellar hand tremor. Both still have white matter abnormalities on MRI and MRS., Conclusions: Elevated lactate in white matter and the possible response to antioxidants suggests mitochondrial dysfunction in progressive spongiform leukoencephalopathy following inhalation of heated heroin vapor.

Granulomatous angiitis of the brain (GAB) is defined histologically by granulomatous inflammation of intracranial blood vessels. We have studied four patients with autopsy-proved GAB who had, respectively, Hodgkin's lymphoma, herpes zoster, neurosarcoidosis, and no associated illness. Headache, fever, or mental change was followed by hemiparesis or quadriparesis, coma, and death in all four patients. There were no diagnostic findings from cerebral computed tomograms, cerebrospinal fluid, or cerebral angiograms; the diagnosis was established only by postmortem examination. Vasculitis was limited to the brain in all four patients, and involved large arteries, small arteries and veins, or both large and small vessels. Differences in etiology and different particulars of the pathologic conditions imply that GAB is a nonspecific reaction, not a unique disease. The diagnosis, moreover, cannot be proved without histologic confirmation. A biopsy specimen is the only way to ascertain the diagnosis in living patients.

In a prospective study, 34 patients with 41 admissions for malignant hypertension were examined by a neurologist before and after antihypertensive treatment. Neurologic examination was normal in 17 cases, and neurologic diagnoses in 19 others included acute cerebral infarction (10), uremic encephalopathy (4), dementia of uncertain etiology (3), multiple infarct dementia (1), and chronic paranoid schizophrenia (1). Five patients had a generalized neurologic syndrome consistent with hypertensive encephalopathy, characterized by altered mental function with or without generalized convulsions. In these patients, neurologic examination was normal 1 to 7 days after lowering of blood pressure, while renal function remained unchanged. Neurologic disease is therefore common in malignant hypertension, and hypertensive encephalopathy may be more frequent than previously described.

Cerebral sarcoidosis was found at autography in a 68-year-old woman with progressive dementia. Of 35 previously reported cases of central nervous system sarcoidosis with dementia, only 1 was over 65 years old, and in only 2 was the presenting clinical syndrome that of a degenerative dementia. Other unusual features of the index case include the restriction of the initial cognitive deficit to memory loss and mild anomia, the scarcity of antemortem evidence for systemic sarcoidosis, a positive tuberculin test, a cerebrospinal fluid (CSF) protein concentration as high as 2028 mg per deciliter. sarcoidosis is a rare but potentially treatable cause of dementia. Consistently normal CSF probably excludes the diagnosis.

A patient is reported in whom a subdural sarcoid granuloma caused symptoms of an intracranial mass lesion, and disappeared following steroid therapy. Twenty-three previous cases with large intracranial sarcoid granulomas have been documented, a review of these 24 cases leads the authors to conclude that: 1) neither symptoms nor nonhistological diagnostic studies, including computerized tomography, differentiate sarcoid from cerebral neoplasms or other central nervous granulomas; and 2) because sarcoid mass lesions frequently respond well to corticosteroid therapy, surgery should probably be reserved for cases in which there is diagnostic uncertainty, a need for emergency decompression, or lack of response to steroids.