Samuel J. Horwitz

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.

Language ability in right and left hemiplegic children

Abstract The language abilities of three right and three left hemiplegic children between 6 and 8 years of age were compared. All children presented pre- or perinatally acquired unilateral lesions confirmed by CT scan, normal hearing, comparable performance I.Q.s, and were being educated in regular classrooms. Right hemiplegic children were found to be inferior to the left hemiplegics on tasks of speech production, vocabulary comprehension, and syntactic comprehension and formulation. Right hemiplegics also acquired single words and two-word combinations later than did left hemiplegics.

A study of hyperlexia

Abstract Twelve children with early intense reading and superior word recognition skills coupled with disordered language and cognitive behavior are described. Cognitive, linguistic, and reading measures evidenced a generalized cognitive deficit in forming superordinate schemata which was not specific to visual or auditory modalities. Positive family histories for reading problems were present for 11 of the 12 children, suggesting a relationship between hyperlexia and dyslexia.

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.

Hyperlexia: Developmental Reading Without Meaning

Children with precocious reading ability despite profound cognitive and language disorders have been described at least as early as 1917, referred to as idiot savants (Parker, 1917; Phillips, 1930) or as a variant of developmental psychoses (Cain, 1969; Goodman, 1972). Initially, referred to as “hyperlexia” by Silberberg and Silberberg (1967, 1968, 1971), several reports have appeared within the past fifteen years further documenting these children’s atypical cognitive and reading development (deHirsch, 1971; Elliott & Needleman, 1976; Goodman, 1972; Huttenlocher & Huttenlocher, 1973; Mehegan & Dreifus, 1972; and Richman & Kitchell, 1981). Recently Healy, Aram, Horwitz and Kessler (1982) studied a group of 12 hyperlexic children concluding that these children present an identifiable syndrome characterized by early and advanced word recognition skills coupled with serious language disorders, especially in comprehending meaning. Further, a strong family history for reading disorders especially in males was demonstrated.

Adverse events following immunization: Assessing probability of causation

The Monitoring System for Adverse Events Following Immunization of the Centers for Disease Control collects data on events temporally related to immunization. Occasionally, reports are received of neurologic disturbances temporally related to receipt of vaccine. Most of these disturbances are events that regularly occur in the absence of immunization. It is then difficult to determine whether the relationship between the immunization and illness is causal or coincidental. We developed a method to assess causation of serious neurologic events by probability theory. By combining epidemiologic information on disease incidence with specific elements of the patient history, an estimate of the odds of vaccine causation can be derived, based on rational assumptions rather than observer bias. The result is not a diagnosis but an estimate of probability.

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.

Neurologic symptoms during coughing paroxysms in cystic fibrosis

Chez 4 patients, on note des troubles de la conscience, des cephalees, des troubles de la vue, des paresthesies, des troubles de la parole et des mouvements anormaux

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

Remote Effect of Malignancy on the Nervous System in Children

A 13‐year‐old boy with Hodgkins 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 Hodgkins 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.