Distribution in time of seizures during presurgical EEG monitoring.

We studied the length of stay needed to record a certain number of seizures in a highly selected group of patients with intractable epilepsy in the final stages of presurgical monitoring. The mean length of stay needed to record one seizure was 2.9 to 3.7 days, depending on the recording technique, 4.5 to 5.5 monitoring days to record three seizures, and 6.1 to 7.6 days to record five seizures. It took 5 days to record at least one seizure in 90% of patients, 7 to 10 days to record three seizures, and 8 to 12 days to record five seizures. One-third of all patients had fewer than five seizures during their stay. Extrapolating from the results in our unit, we computed a need for 91 to 227 presurgical epilepsy monitoring beds to evaluate 2,000 to 5,000 similar epilepsy surgery candidates requiring invasive monitoring in the United States each year. Additional beds would be needed to monitor patients admitted for other purposes.

Fleck retina in Kjellin's syndrome.

We examined members of two families with Kjellin's syndrome, an autosomal recessive disorder characterized by spastic paraparesis, dementia, and macular changes. Although the retinal lesions resemble the flecks of fundus flavimaculatus by ophthalmoscopy, they appear distinct from previously reported retinal flecks by fluorescein angiography.

The current status of neurology of family practice residency programs.

The status of neurology in family practice residency programs was determined. Of 373 programs surveyed by questionnaire, 266 responded (71.3%). Neurology is a required rotation in 48% of the programs, most frequently done during the second year of residency. Preceptors are mainly neurologists in private practice. There is a need for cooperation between neurologists and family practitioners to develop appropriate educational experiences for family practice residents.

Confidence bands for the growth of head circumference in achondroplastic children during the first year of life.

Confidence bands are established for the growth of head circumference in achondroplastic children during their first year of life. the model of (formula see text), where a = (1, t,t2) and t is aged expressed in months provides the best estimated population mean curve with 95% confidence limits.

The malignancy of dementias.

Sruvival times were determined for 982 patients admitted over a ten-year period to the Geneva Psychiatric Clinic. Patients with dementia had one-third the life expectancy of controls. Among patients classed as having Alzheimer disease, the longest survival times were in those with neurofibrillary tangles involving the neocortex, while those lacking this anatomical abnormality had the shortest survival times. Except for women with Alzheimer disease, patients with dementia had less than 10% life expectancy from the time of their admission to the clinic compared to the life expectancy of Geneva's population.

Exacerbation generalized nonconvulsive seizures with ethosuximide therapy.

We describe a patient whose absence seizures increased greatly in number after the addition of ethosuximide to ongoing phenobarbital therapy. This unusual response and review of other cases in the literature suggested the possibility of some as yet underfined interaction between ethosuximide and other anticonvulsant drugs. That a particular subytpe of epilepsy may be worsened by ethosuximide therapy is also considered.

Atlantoaxial malformation in a 46,XY, 17q+ child.

A child with 46,XY, 17q+ chromosome aberration and unusual skeletal abnormalities had the presenting symptom of respiratory arrest after a fall and minor head trauma. He exhibited proportionate short stature, peculiar facies with antimongoloid slant, simian creases, postaxial hexadactyly, metatarsus adductus deformity, and anomalous penoscrotal configuration. Delayed speech and mild mental retardation were also present. Atlantoaxial dislocation secondary to odontoid process hypoplasia was demonstrated. Additional skeletal abnormalities included dysplastic changes in the first and fourth metacarpals, the middle phalanx of the second finger, and hypoplasia of the first metatarsal, with medial insertion of the great toe. Treatment consisted of halo jacket cast and fusion of the occiput to C1, C2, and C3. Early detection and preventive surgery for atlantoaxial dislocation is necessary to prevent neurological deficit and possibly death by respiratory failure.

Specificity of the clinical diagnosis of dementia.

The specificity of anatomo-clinical observations were investigated on 776 out of 982 consecutive persons hospitalized at the University Psychiatric Clinic of Geneva. Discriminant function analysis shows that most of the anatomical classes (no dementia, senile dementia, Alzheimerized senile dementia and Alzheimer's presenile dementia, vascular dementia, combined dementia nnd undefined form of encephalopathy) are at least partially separable (less than 50% overlap). On the basis of anatomical criteria, Alzheimer's presenile dementia is not separable from Alzheimerized senile dementia, and senile dementia is not separable from combined dementia. Differentiation between the anatomical classes is improved by a preliminary analysis based on clinical diagnosis. Senile plaques account for 43.4% of the total variation between the anatomical classes. The coefficient of agreement between anatomical and clinical diagnosis is 0.27, which is highly significant. The diagnosis of senile dementia has a poor specificity, while the diagnosis of senile Alzheimerized dementia has a better one. The diagnosis of combined dementia has to be reserved for cases with a similar intensity of vascular and degenerative changes. Alzheimer's presenile dementia has a distinct dominant hereditary pattern and must be considered a separate entity. The high specificity of the diagnosis of Alzheimer's presenile dementia makes it possible to conduct epidemiologic and genetic surveys based on clinical data.

Craniopagus twins.

Craniopagus twins are described. At the age of 24 years, the twins appeared to have adapted well to their disability. The site of union, their relatively advanced age, and the high risk of operation were the reasons for not proposing surgical separation. Perceptive upbringing and adaptation to the disability may be alternatives to the neurosurgical management of this birth defect.