Conditions associated with Huntington's disease at death. A case-control study.

To identify conditions associated with reduced survival in patients with Huntington's disease, we studied all 1978 US death certificates on which Huntington's disease (331.0 in the International Classification of Diseases, eighth revision) was listed. For each of the 495 cases identified, two control deaths were matched by age, race, sex, county, and year of death. Pneumonia, choking, nutritional deficiencies, and chronic skin ulcers were increased in cases relative to controls.

Syphilitic polyradiculopathy in an HIV-positive man.

We report a case of syphilitic lumbosacral polyradiculopathy in an HIV-positive, 22-year-old bisexual man with a recent history of secondary syphilis treated with intramuscular penicillin. He presented with rapidly progressive pain and weakness, and muscle wasting in the legs. CSF was under increased pressure and showed a marked pleocytosis (1,130 cells/mm3), hypoglycorrhachia (19 mg/dl), and very elevated protein (1,000 mg/dl). Serum and CSF VDRL serologies were positive. In the legs, nerve conduction studies and needle EMG were consistent with an asymmetric lumbosacral polyradiculopathy with active denervation. His clinical state, CSF, and electrophysiologic studies all improved promptly and markedly after intravenous penicillin. This report documents an uncharacteristically aggressive case of neurosyphilis accompanied by marked changes in the CSF in an HIV-positive patient. While the immunologic effects of HIV and syphilis in combination are not yet fully understood, the cellular immunity defect associated with HIV may alter the natural history of syphilis in patients with concomitant infection, producing unusually aggressive forms or atypical presentations of neurosyphilis.

Huntington's disease mortality in the United States.

The first analysis of multiple cause-of-death data for Huntington's disease in the United States was performed, using data from the National Center for Health Statistics for the period 1971 and 1973 through 1978. The overall mortality rate was 2.27 per million population per year, approximately 80% higher than the corresponding rate for deaths in which Huntington's disease was listed as the underlying cause of death. Age-specific mortality rates peaked around age 60. Rates were similar for both sexes, and higher in whites than nonwhites. Age-adjusted rates were similar for United States whites and reported values from the Scandinavian countries. The leading causes of death were pneumonia and heart disease. Other common causes of death were nutritional deficiencies; mental disorders; cerebrovascular disorders; and accidents, poisonings, and violence. Suicide was rarely reported.

A population-based study of neonatal seizures in Fayette County, Kentucky.

This population-based, retrospective cohort study of neonatal seizures included all neonates born to residents of Fayette County, Kentucky, from 1985 to 1989. We ascertained potential cases by computer search of hospital-based medical record systems, Kentucky Center for Health Statistics birth certificate data files, and National Center for Health Statistics multiple-cause-of-death mortality data files. Medical records for potential cases were abstracted, and relevant portions were reviewed independently by three neurologists using prospectively determined case-selection criteria. Seizures occurred in 58 of 16,428 neonates (3.5/1,000 live births). An additional 15 neonates had possible seizures, for a combined risk of 4.4/1,000 live births. Neonatal seizure risk varied inversely with birth weight: 57.5/1,000 live births among very low birth weight infants (< 1,500 grams) compared with 4.4/1,000 for infants with moderately low birth weight (1,500 to 2,499 grams), 2.8/1,000 for those with normal birth weight (2,500 to 3,999 grams), and 2.0/1,000 for those with high birth weight (4,000 or more grams). Risk varied among the four hospitals in the county with obstetric units, the university hospital having the highest risk. Risk did not differ by race or gender. A Cox proportional hazards model confirmed the results of the simpler univariate analyses. Differences in birth weight of the subpopulations served by each hospital accounted for much but not all the differences in hospital-specific risk.

Magnetic resonance imaging in cervical cord birth injury.

Three children who sustained cervical cord injury due to birth trauma are described. The clinical and radiological data, and the central nervous system pathology of one child, illustrate the advantages and feasibility of magnetic resonance imaging in ventilator-dependent children with cervical cord lesions.

Late-onset sporadic progressive subcortical gliosis.

We report two sporadic cases of progressive subcortical gliosis (PSG) with onset after age 60. The presentation included slowly progressive dementia with memory loss, geographic disorientation, and personality change. Both were diagnosed clinically as Alzheimer's disease (AD) and both met NINCDS-ADRDA criteria for probable AD. Autopsy revealed generalized atrophy, predominantly involving the white matter of the frontal and temporal lobes. Microscopically, prominent fibrillary astrocytosis was present in the subcortical white matter and in the subpial and deep layers of the overlying cerebral cortex. Mild cortical neuron loss accompanied the gliosis, but no myelin loss was evident. Amyloid deposits and neuronal cytoskeletal inclusions were absent.

Klüver-Bucy syndrome in juvenile neuronal ceroid lipofuscinosis.

Although Klüver-Bucy syndrome in adults is commonly associated with neurodegenerative conditions, Klüver-Bucy syndrome in children has been recognized almost exclusively in association with acute bitemporal injury or dysfunction. We report a child with juvenile neuronal ceroid lipofuscinosis, who developed dementia, childhood-onset autistic disorder, and Klüver-Bucy syndrome. The behavioral features of this case are compared with those of previously reported cases of juvenile neuronal ceroid lipofuscinosis and with previous reports of Klüver-Bucy syndrome in children and adults.

Visual "release" hallucinations in juvenile neuronal ceroid-lipofuscinosis.

A child with juvenile neuronal ceroid-lipofuscinosis developed formed and unformed visual hallucinations with the development of blindness. The hallucinations lasted for prolonged periods, were of simple, varied, and novel content and were not associated with any ictal manifestations. The clinical features support a "release" mechanism for the hallucinations.

Presentation, clinical course, and outcome of childhood stroke.

We reviewed the presentations, clinical courses, and outcomes of 42 children with unilateral hemispheric stroke. Infants with strokes identified within the first few days of life usually presented with seizures. These infants had few abnormal neurologic findings as neonates, but hemiparesis became evident as gross motor development proceeded. Infants with strokes identified later in the first year of life usually presented with pathologic early hand preference without a history of an ictus. During subsequent development, the motor deficits in these children became more evident, producing an apparent progression of the neurologic abnormalities. Strokes identified in older children typically presented as sudden hemiparesis, often associated with seizures. The hemiparesis in these children was most severe at the onset, followed by some improvement in strength in all patients. Functional outcome was variable. At last follow-up, all children were ambulatory, some with clinically apparent hemiparesis. Eight of the 42 children (19%) developed recurrent seizures with an onset ranging from 4 months to more than 10 years (median: 26 months) after the stroke.

Interobserver variability in the classification of neonatal seizures based on medical record data.

This population-based, retrospective cohort study of neonatal seizures included all 16,428 neonates born to residents of Fayette County, Kentucky, from 1985 to 1989. Eighty potential cases were ascertained by computer search of hospital-based medical record systems, birth certificate data files, and multiple-cause-of-death mortality data files. Medical records for potential cases were abstracted, and relevant portions were reviewed independently by three neurologists using prospectively determined criteria. Both unweighted and weighted kappa statistics were used to measure agreement between each pair of observers in the classification of potential cases as seizures, possible seizures, or not seizures, adjusting for the proportion of agreement expected by chance. Agreement in the classification of potential cases was excellent (kappa = 0.72-0.79, average = 0.76; weighted kappa = 0.85-0.88, average = 0.87). The kappa extension statistic of Kraemer was used to assess agreement in the classification of seizure types by a simplification of the classification scheme of Volpe. This documented excellent agreement between raters in the classification of seizure types (kappa e = 0.72). Experienced raters can reliably classify potential cases of neonatal seizures using seizure descriptions transcribed from medical records.