PubMed Health⌕ Search

Biomedical subjects

S J Horwitz

Publications and source records attributed to S J Horwitz.

At least 19 recordsLinked to original sources
PubMed1995

Opsoclonus myoclonus syndrome secondary to Epstein-Barr virus infection.

We report the first two children to have the "opsoclonus myoclonus syndrome" after a serologically confirmed acute Epstein-Barr virus infection. Although opsoclonus was absent in one patient, both patients had all other features typical of this syndrome. Patients without opsoclonus have been described as having an isolated postinfectious myoclonus syndrome but probably lie within the spectrum of opsoclonus myoclonus syndrome. The prognosis for the opsoclonus myoclonus syndrome in children with Epstein-Barr virus infection and without evidence for a neuroblastoma appears to be better than in those patients where a cause cannot be identified.

Antibodies, Viral↗
PubMed1994

Deceptively normal MR in early infantile Krabbe disease.

The authors present two cases of 5-month-old children with early infantile Krabbe disease studied by CT and MR. Both infants had characteristic CT scans for the disease consisting of symmetric hyperdensity involving the cerebellum, thalami, caudate, corona radiata, and brain stem. One of the infants had a deceptively normal initial MR examination, with dramatic progression of the white-matter disease over the following 4 months.

Brain↗
PubMed1990

Congenital absence of the corticospinal fibers: pathologic and clinical observations.

We describe 2 patients with primary absence of corticospinal tracts. Both presented with delayed motor development, head lag, and spasticity of the limbs. We could not identify precentral cortex, and there were no corticospinal fibers below the internal capsules. We postulate that the fibers were displaced or interrupted early in their development.

Brain↗
PubMed1989

Language and motor findings in benign megalencephaly.

9 children between the ages of 5 and 12 yr. whose head circumferences were greater than the 98th percentile and showed negative CT scans and histories for disease associated with increased brain size were studied. A battery of language, articulation, intelligence, and motor tests were administered to subjects and in each case to a sibling whose head circumference was normal. Analysis showed few differences in intelligence and language comprehension between the megalencephalic children and their siblings. Significant differences were observed in motor proficiency, with over 50% of the megalencephalic children scoring below the 10th percentile for their age group. Three megalencephalic children displayed articulation errors. All subjects had at least one parent whose head circumference exceeded the 98th percentile. These results suggest a relationship between benign megalencephaly and developmental motor difficulties.

Cephalometry↗
PubMed1985

Motor neuron disease and adult hexosaminidase A deficiency in two families: evidence for multisystem degeneration.

We studied three patients from two unrelated families with adult hexosaminidase A deficiency. A 30-year-old, non-Jewish proband in the first family had juvenile amyotrophic lateral sclerosis that evolved to mild dementia, ataxia, and axonal (neuronal) motor-sensory peripheral neuropathy. A 36-year-old Jewish proband in the second family had "pure" spinal muscular atrophy. One supposedly healthy brother of the first proband was found to have borderline IQ, mild spasticity, and ataxia but no evidence of motor neuron disease. Marked cerebellar atrophy was detected by head scans in all three patients. In both probands electromyograms were characterized by prominent, complex repetitive discharges in many muscles. Hexosaminidase A activities against the artificial substrate were similar to those reported in infantile Tay-Sachs disease; however, the hexosaminidase A level against GM2 substrates was higher than that found in infantile Tay-Sachs disease. The hexosaminidase A levels of the parents were in the heterozygous range. Motor neuron disease in our patients and in those previously described appears to be part of a multisystem degeneration of the nervous system.

Adult↗
PubMed1983

Sequential and non-speech praxic abilities in developmental verbal apraxia.

Verbal and non-verbal sequential abilities were compared in a group of 10 children with developmental verbal apraxia. Manual gestures, constructional praxic abilities and non-speech volitional oral movements were also studied. Results indicated that these children were deficient in verbal sequential abilities, both with reference to their non-verbal sequential abilities and to normative data. As a group, the children did not present manual-gestural or constructional apraxias, nor oral apraxias for single non-speech volitional movements. However, most had difficulty with sequences of non-speech volitional oral movements.

Adolescent↗
PubMed1983

Histidinemia. A case with resolution of myoclonic seizures after treatment with a low-histidine diet.

An infant with histidinemia had a myoclonic seizure disorder that was unresponsive to therapy with anticonvulsants. The seizures stopped promptly after a histidine-restricted diet was instituted. This case suggests that in some individuals with histidinemia, diet therapy may be effective. It is not known what distinguishes this case from previously reported cases in which diet therapy was not effective.

Amino Acid Metabolism, Inborn Errors↗
PubMed1981

Response of lymphangiectasis to radiotherapy.

A 14-year-old girl with lymphangiectasis of the skull causing rapid extensive destruction of the left orbit, zygoma, mandible, sphenoid, and occiput underwent radiotherapy with 2000 rad to the entire skull, mandible, and upper cervical vertebrae. Three years later, progression of the disease has ceased, and the involved bone is slowly remineralizing.

Adolescent↗
PubMed1980

Bilateral carpal tunnel syndrome in an adolescent.

A 13-year-old boy with nocturnal paresthesias of the hands was shown to have bilateral carpal tunnel syndrome by electrodiagnosis. Surgical findings included thickening of the transverse carpal ligament and local anomalies of tendon and muscle. Surgical correction produced prompt relief.

Adolescent↗
PubMed1980

Cerebral herniation in bacterial meningitis in childhood.

Among 302 infants and children with acute bacterial meningitis, the syndrome of cerebral herniation occurred in 3 of 10 fatal cases and in 15 patients who survived. The interval from time of admission to herniation was 8 hours or less, and no predictive factors could be determined. Seizures occurring immediately prior to cerebral herniation made diagnosis more difficult. Early recognition of cerebral herniation and prompt treatment with osmotic diuretics can reduce the mortality of bacterial meningitis.

Acute Disease↗
PubMed1980

Remote effect of malignancy on the nervous system in children.

A 13-year-old boy with Hodgkin's disease developed acute polyneuropathy and autoimmune hemolytic anemia. Sural nerve biopsy and postmortem examination demonstrated no metastatic involvement and a lack of cellular inflammatory infiltration in the nervous system. There was significant axonal degeneration in peripheral nerves and the dorsal funiculus. These findings suggest a non-metastatic polyneuropathy associated with Hodgkin's disease, and similar cases, predominantly in adult patients, have been reported. This case emphasizes the occurrence of a paraneoplastic syndrome in children, though such syndromes appear to be rare compared with adults.

Adolescent↗