ABSTRACT
We report here a family in which 4 out of 11 sibs of unrelated parents showed the typical clinical features of Dejerine-Sottas disease. Sensory disturbance was present in only one case and age at onset was delayed to 4 years in another. The others all conformed to the infantile form of the disease. This is the first time in which mental retardation has been reported in this disorder.
Subject(s)
Hereditary Sensory and Motor Neuropathy/complications , Intellectual Disability/complications , Adolescent , Atrophy , Child , Electromyography , Female , Fingers/abnormalities , Hereditary Sensory and Motor Neuropathy/diagnosis , Hereditary Sensory and Motor Neuropathy/genetics , Humans , Male , Muscles/pathology , Pedigree , Toes/abnormalitiesSubject(s)
Developing Countries , Ethnicity/statistics & numerical data , Parkinson Disease/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Cross-Sectional Studies , Female , Humans , Incidence , India/epidemiology , Infant , Iran/ethnology , Male , Middle Aged , Parkinson Disease/etiology , Parkinson Disease/geneticsABSTRACT
We carried out a door-to-door survey to screen for neurologic diseases, including peripheral neuropathy, in a community of 14,010 Parsis living in housing colonies in Bombay, India. The most common neurologic disorder was peripheral neuropathy with 334 cases (2,384 cases/100,000 population). The most common neuropathy was compressive, with diabetes the most common noncompressive etiology. There was no leprosy, and nutritional neuropathies were rare.
Subject(s)
Peripheral Nervous System Diseases/epidemiology , Age Factors , Humans , India/epidemiology , Iran/ethnology , Middle Aged , Nerve Compression Syndromes/classification , Nerve Compression Syndromes/complications , Peripheral Nervous System Diseases/ethnology , Prevalence , Religion , Sex FactorsABSTRACT
A door-to-door survey was carried out to screen a community of 14,010 people (Parsis living in colonies in Bombay, India) for possible neurological diseases. High school graduates, social workers, and a medical student administered a screening questionnaire that, in a pilot study, had a high sensitivity for identifying febrile seizures in children under the age of 14 years. Neurologists used defined diagnostic criteria to evaluate individuals positive on the screening survey. There were 1,581 children under the age of 14 years. Twenty-eight children (19 boys, 9 girls) suffered from febrile seizures (17.7/1,000 population). The present prevalence study is the first from India and suggests that, contrary to expectation, the frequency of febrile seizures may be no different in developing and developed countries.
Subject(s)
Seizures, Febrile/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , India/epidemiology , Infant , Male , Middle Aged , Pilot Projects , PrevalenceABSTRACT
This is the first report from India of a family of 4 sisters and 3 brothers in whom the proband showed a complete form of the choreo-acanthocytosis syndrome, whereas the 2 brothers who had died earlier of this disease had no examination of their blood. The apparently healthy members of this family also showed acanthocytosis, suggesting that this is a multisystem disorder affecting cell membranes.
Subject(s)
Acanthocytes/metabolism , Erythrocytes, Abnormal/metabolism , Hematologic Diseases/metabolism , Lipoproteins/deficiency , Female , Hematologic Diseases/genetics , Humans , Middle AgedABSTRACT
A door-to-door survey was carried out to screen a community of 14010 people (Parsis living in colonies in Bombay, India) for possible neurologic diseases. High school graduates, social workers, and medical students administered a screening questionnaire that had been shown in a pilot survey to have a sensitivity of 100% for identifying those with Parkinson's disease. Neurologists used defined diagnostic criteria to evaluate individuals positive on the screening survey. There were 46 people (25 men, 21 women) who suffered from Parkinson's disease (328.3 cases per 1000 population). The age-specific prevalence ratios increased consistently with age. Age-adjusted prevalence ratios were slightly higher for men.
Subject(s)
Parkinson Disease/ethnology , Age Factors , Aging , Cross-Sectional Studies , Female , Humans , India , Male , Parkinson Disease/epidemiology , Sex Factors , Tremor/epidemiology , Tremor/ethnologyABSTRACT
A door-to-door survey for neurologic diseases was conducted in a community of 14,010 people (Parsis living in colonies in Bombay, India). Neurologists used defined diagnostic criteria to evaluate persons with positive results on the screening survey. Two hundred thirty-three people (104 men; 129 women) were identified as having essential tremor. The overall prevalence ratio was 1663.1 [corrected] per 100,000 population. Age-specific prevalence ratios increased with age. Age-adjusted prevalence ratios were similar for men and women. To the best of our knowledge, this is the first community-based survey for essential tremor in Asia.
Subject(s)
Tremor/ethnology , Adolescent , Adult , Aged , Aged, 80 and over , Female , Health Surveys , Humans , India , Iran/ethnology , Male , Mass Screening , Middle Aged , Religion , Tremor/diagnosisABSTRACT
We carried out a door-to-door-survey to screen a community of 14,010 people (Parsis living in colonies in Bombay, India) for possible neurologic diseases, and used defined diagnostic criteria to evaluate people who tested positive on the screening survey. There were three clinically definite cases of multiple sclerosis (21/100,000). This is the first prevalence survey for multiple sclerosis in a large developing country.
Subject(s)
Multiple Sclerosis/ethnology , Humans , India , Iran/ethnology , Multiple Sclerosis/epidemiology , Religion , United StatesABSTRACT
A door-to-door survey was carried out to screen a community of 14,010 people (Parsis living in colonies in Bombay, India) for possible neurologic diseases. High school graduates, social workers, and medical students administered a screening questionnaire that in a pilot survey had a sensitivity of 100% for identifying persons with epilepsy. Neurologists used defined diagnostic criteria to evaluate individuals positive on the screening survey. Sixty-six persons (43 males, 23 females) suffered from epilepsy (4.7 cases/1,000). Of those, 50 (34 males, 16 females) had active epilepsy (3.6 cases/1,000). The age-specific prevalence ratios remained fairly constant for each age group except for a small peak in the group aged 20-39 years for all epilepsy cases combined. Age-adjusted prevalence ratios were higher for males. The most common seizure type was partial (36 cases). The most frequently associated conditions were cerebral palsy and mental retardation. The majority of individuals were receiving medication as of prevalence day (47 cases).
Subject(s)
Epilepsy/epidemiology , Ethnicity , Adolescent , Adult , Age Factors , Aged , Anticonvulsants/therapeutic use , Child , Child, Preschool , Cross-Sectional Studies , Epilepsy/drug therapy , Epilepsy/prevention & control , Female , Health Surveys , Humans , India , Infant , Infant, Newborn , Male , Middle Aged , Sex FactorsABSTRACT
All eligible adult cases of completed ischemic stroke (N = 112) derived from a prevalence survey of the Parsi community in Bombay, India, served as the basis of a case-control study of risk factors. The stroke morbidity rate in this group was similar to that reported for North America. A control subject free of stroke was selected randomly from the same population and matched to each case by age and sex. Information was obtained on various risk factors. Hypertension, both by history (odds ratio = 10.8) and by examination (odds ratio = 2.1), had a statistically significant association with stroke (p less than 0.01). This is one of the first community-based case-control studies of stroke in a large developing country.
Subject(s)
Brain Ischemia/ethnology , Cerebrovascular Disorders/ethnology , Adult , Brain Ischemia/complications , Cerebrovascular Disorders/etiology , Child , Child, Preschool , Humans , India , Iran/ethnology , ReligionABSTRACT
A door-to-door survey of 14,010 Parsis living in colonies in Bombay, India, screened people for possible neurologic diseases. High school graduates, social workers, and a medical student administered a questionnaire that had been shown in a pilot study to have a sensitivity of 100% for identifying persons with stroke. Neurologists used defined diagnostic criteria to evaluate those who were positive on the screening survey. One hundred eighteen persons (57 men, 61 women) suffered from stroke (842.3 cases/100,000 population). The age-specific prevalence ratios increased with age for both sexes and for each age group. Age-adjusted prevalence ratios were slightly higher for men than for women. The most common type of stroke was ischemic (114 cases).
Subject(s)
Cerebrovascular Disorders/epidemiology , Adolescent , Adult , Aged , Child , Child, Preschool , Female , Humans , India , Infant , Infant, Newborn , Male , Middle AgedABSTRACT
There is a paucity of trained neurologists in developing countries. We designed a questionnaire to rapidly screen a community of 851 people (Parsis living in a colony in Bombay, India) for possible neurologic diseases. This questionnaire was pretested and found to have a sensitivity of 100 percent for detecting epilepsy, febrile seizures (only in children), completed stroke, peripheral neuropathy, movement disorders, cerebral palsy, mental retardation, and severe dementia. The screening questionnaire was administered by trained lay health workers. One hundred and sixty-three people were identified by this questionnaire as possibly having neurologic disease. Neurologists later examined these 163 people and found that 80 of them actually suffered from at least one of the neurologic diseases of interest (positive predictive value = 48 percent). The most common neurologic disorders were peripheral neuropathy (32 cases), essential tremor (13 cases), stroke (12 cases), Parkinson's disease (six cases), and epilepsy (four cases).
Subject(s)
Nervous System Diseases/epidemiology , Adolescent , Adult , Aged , Community Health Workers , Ethnicity , Female , Humans , India , Male , Middle Aged , Pilot Projects , Population Surveillance , Sensitivity and Specificity , Surveys and QuestionnairesSubject(s)
Accidents, Occupational , Disasters , Gas Poisoning/complications , Isocyanates , Nervous System Diseases/chemically induced , Adult , Child , Cyanates/poisoning , Female , Follow-Up Studies , Humans , India , Male , Middle AgedABSTRACT
Machado-Joseph-Azorean (MJA) disease is an autosomal-dominant multisystem motor degeneration (with cerebellar ataxia as an important manifestation) that is seen mainly in people of Portuguese descent. Recently, a family in Japan with probable MJA disease has been described. We describe a family with clinical features similar to those of MJA disease; these are the first cases to be described in India. Further pathologic study is necessary to confirm the diagnosis in our cases. Meanwhile, further search for such cases in India should yield interesting genetic clues to the disease.
