Nicotine exposure and Parkinson disease.

The observed inverse relationship between smoking and Parkinson disease has prompted suggestions that nicotine, a centrally active agent, might protect against the disease. In this case-control study, cases were found to have ever regularly smoked cigarettes significantly less frequently than sex-, race-, and age-matched neighbors. This report analyzes the detailed smoking histories of cases and neighbors to see if these histories support the nicotine protection hypothesis. Estimated nicotine exposure before age at onset of symptoms for smoking cases was 186.1 g; for smoking controls it was 208.3 g (p = 0.34). Among the cases, severity of disease was not related to the extent of nicotine exposure before disease onset. Age at onset of symptoms for smoking cases (52.7 years) was not delayed (57.8 years for nonsmoking cases). Since the study was unable th find further support for the nicotine protection hypothesis, it is concluded that the observed inverse relationship between smoking and Parkinson disease is likely explainable by other factors, such as selective mortality or pre-morbid behavioral and/or constitutional changes.

Cigarette smoking and Parkinson disease: 1. Comparison of cases with matched neighbors.

In previous studies, there were fewer cigarette smokers among persons with Parkinson disease than among other patients. We reinvestigated this phenomenon, using nonpatient controls. In home interviews with 237 Parkinson patients and 474 age-, sex-, and race-matched neighbors, we inquired about consumption of tobacco, coffee, tea, and alcohol. All Parkinson patients were diagnosed by a neurologist, had two or more cardinal features of parkinsonism, and had not received chronic phenothiazine therapy. One hundred fifty (63%) of 237 cases and 224 (47%) of 474 controls never smoked cigarettes (p < 0.0001). Significantly different smoking rates were also preset at 10 and 20 years before the onset of parkinsonism.

Acute hemiplegia of childhood.

Acute hemiplegia of childhood is briefly reviewed with examples of various etiologies. The role of brain scans in diagnosing and following children with this syndrome is emphasized. Cerebral angiography and computer tomography represent complementary examinations in the evaluation of these patients.

Huntington disease associated with Alzheimer disease.

The clinicopathological study of a case of Huntington disease with early severe dementia is presented. The pathological findings were those of Huntington disease (atrophy with neuronal loss and astrocytosis in the neostriatum) and Alzheimer disease (neuronal loss with numerous senile plaques and neurofibrillary tangles in the neocortex). Ultrastructural study of neurofibrillary tangles showed that they were composed of twisted tubules similar to those found in Alzheimer disease.

Hemiballismus-hemichorea treated with dimethylaminoethanol.

An elderly lady with subacute onset of disabling hemiballismus experienced sustained remission on dimethylaminoethanol 150 mg/day. Two exacerbations followed interruption of therapy. Remission followed resumption of therapy. No undesirable side effects occurred with this treatment. A lady age 85 experienced a very favorable sustained reduction of hemiballismus when treated with dimethylaminoethanol (DMAE or Deanol). Her age and hypertension made the risk of thalamotomy unacceptably high. DMAE was tried because of reports of reduction of chorea, and other dyskinesias with this drug.

Late-infantile neuronal ceroid-lipofuscinosis. An ultrastructural study of lymphocyte inclusions.

Ultrastructural study of peripheral blood from two patients with late-infantile neuronal ceroid-lipofuscinosis with curvilinear bodies, demonstrated in brain biopsies revealed curvilinear bodies in lymphocytes. These findings indicate that ultrastructural investigation of circulating lymphocytes may be useful in the diagnosis of late-infantile neuronal ceroid-lipofuscinosis and may circumvent the need for brain biopsy. Curvilinear bodies were also present in the lymphocytes of a neurologically normal younger female sibling of patient 1, who has had a single seizure, leading to speculation that she may be in the early stages of the same disease. This case suggests the possibility of early detection of this disorder and indicates the importance of screening lymphocytes of siblings of affected patients. Tubular cytoplasm inclusions were present in a high percentage of lymphocytes of the asymptomatic parents and a younger sibling of these patients.

Acetoxycycloheximide enhances audiogenic seizures in DBA-2J mice.

Inborn errors of metabolism often cause epilepsy, as with certain strains of mice. Aggravating the metabolic defect with a protein synthesis inhibitor increases the symptoms. Mature animals that have "outgrown" their genetic susceptibility to audiogenic seizures are made susceptible again by acetoxycycloheximide. After a single small dose the incidence and severity of audiogenic seizures increases at 16 hours, reaches a maximum of 40 hours, and then declines gradually.