The prevalence of Parkinson's disease in an Arab population, Wadi Ara, Israel.

BACKGROUND: The prevalence of Parkinson's disease varies among ethnic and geographic groups around the world, being very low in China and high in Argentina. While the main etiology of the disease has yet to be determined, environmental, occupational and genetic factors seem to play important roles. OBJECTIVES: To estimate the prevalence of PD in an Arab Muslim population in Israel, using the drug tracer approach. METHODS: We studied a Muslim Arab population living in a well-defined geographic area in Israel, with the majority located in two towns and two large villages. Of the approximately 115,000 residents, about 38% are under the age of 15 and 7.75% are older than 65. Drug tracer methodology was applied in this study. All those who were on anti-PD medication were identified and examined by a neurologist to confirm the diagnosis. RESULTS: The overall crude prevalence of PD in this population was low, 43.24/100,000, while the prevalence in the age group above 65 years was 477.32/100,000. Below this age, the prevalence was very low, 12.29/100,000. PD prevalence was higher in males than in females (ratio 1.17); 63% of male patients smoked cigarettes. The prevalence was found to be twice as high among the residents of rural areas, where most inhabitants work in agriculture. CONCLUSIONS: The prevalence of PD among the Arab population in Israel is considered low and comparable to that reported in other Arab countries.

Huntington disease in subjects from an Israeli Karaite community carrying alleles of intermediate and expanded CAG repeats in the HTT gene: Huntington disease or phenocopy?

We report a cluster of patients from a Karaite Jew community with a movement disorder suggestive of Huntington disease (HD), in some cases associated with repeat lengths below the edge of 36 CAG repeats. The study describes the clinical and genetic features of four patients who were followed over several years. Patients belonged to an inbred family in whom progressive chorea, manifesting predominantly with dystonia and cerebellar features, developed during middle age. Although severe psychiatric symptoms ultimately developed in two of the four patients, cognitive function remained reasonably well preserved in all of them even after several disease years. Moderate cognitive deficits were limited to the visuomotor organization and abstract thinking subtests in three of the four patients. Qualitative brain imaging showed atrophy of brain predominantly involving cortex and cerebellum. Genetic testing revealed a variable mutation penetrance among family members, some affected members showing an upper allele size ranging from 34 to 49, whereas others remained unaffected despite the presence of the full mutation beyond 40 CAG repeats. Co-morbidity with recessive hereditary inclusion body myopathy was found in two subjects from one family. Although the main diagnosis of HD remains to be confirmed by further neuropathological studies, these cases may suggest that HD could manifest with as few as 34 CAG repeats, in some geographic areas, the disease phenotype most probably being influenced by additional, as yet unidentified, genes.

Acetylcholinesterase/paraoxonase interactions increase the risk of insecticide-induced Parkinson's disease.

Exposure to agricultural insecticides, together with yet incompletely understood predisposing genotype/phenotype elements, notably increase the risk of Parkinson's disease. Here, we report findings attributing the increased risk in an insecticide-exposed rural area in Israel to interacting debilitating polymorphisms in the ACHE/PON1 locus and corresponding expression variations. Polymorphisms that debilitate PON1 activity and cause impaired AChE overproduction under anticholinesterase exposure were strongly overrepresented in patients from agriculturally exposed areas, indicating that they confer risk of Parkinson's disease. Supporting this notion, serum AChE and PON1 activities were both selectively and significantly lower in patients than in healthy individuals and in carriers of the risky polymorphisms as compared with other Parkinsonian patients. Our findings suggest that inherited interactive weakness of AChE and PON1 expression increases the insecticide-induced occurrence of Parkinson's disease.

Epilepsia partialis continua possibly caused by cerebellar lesion.

The cerebellum is known to have inhibitory effects on seizures. Nevertheless, cerebellar dysplastic lesions can be epileptogenic. We report a patient presenting with epilepsia partialis continua (EPC) following a hemorrhagic cerebellar lesion. Possible pathophysiological mechanisms are discussed.

Facial paralysis and meningitis caused by Rickettsia typhi infection.

Involvement of cranial nerves was rarely reported in various rickettsioses. We present here for the first time a case of peripheral facial paralysis associated with Rickettsia typhi infection.

Spontaneous supratentorial intracerebral hemorrhage. Criteria for short-term functional outcome prediction.

BACKGROUND AND PURPOSE: To identify the significant prognostic factors, upon admission, and construct a set of criteria to predict short-term functional outcome of patients with intracerebral hemorrhage (ICH). METHODS: The records as well as the radiological findings, of 184 consecutive cases of spontaneous supratentorial intracerebral hemorrhage, that were treated medically, were reviewed. The hemorrhage was graded according to size, mass effect and intraventricular extension. Outcome upon discharge was scored using the modified Rankin Scale. A score of four or more was considered a poor outcome. Multivariate analysis was used to identify the factors associated with a poor outcome. RESULTS: Six significant and independent prognostic variables were identified: decreased level of consciousness, severe hemiparesis, age older than sixty, large hematoma size, midline shift and intraventricular extension on CT. These variables were scored systematically to produce the ICH criteria. The sum of these criteria yields a figure between zero and six termed ICH score. Patient grouping according to ICH score identified four distinctive, prognostic groups: I - score of zero to one, II - score of two, III - score of three and IV - score of four to six in which 82%, 53.7%, 23.3% and 0% achieved a good outcome respectively (P < 0.05 for all groups). CONCLUSIONS: The present study identified six independent admission criteria predicting the short-term functional outcome of ICH patients. Their sum may serve to predict the short-term functional outcome upon admission of medically-treated patients with spontaneous supratentorial intracerebral hemorrhage.