Robert V. Groover

SEIZURES AFTER HEAD TRAUMA: A POPULATION STUDY

A cohort of 2747 patients with head injuries was followed for 28,176 person-years to determine the magnitude and duration of the risk of posttxaumatic seizures. Injuries were classified as severe (brain contusion, intracerebral or intracranial hematoma, or 24 hours of either unconsciousness or amnesia), moderate (skull fracture or 30 minutes to 24 hours of unconsciousness or amnesia), and mild (briefer unconsciousness or amnesia). The risk of posttraumatic seizures after severe injury was 7.1% within 1 year and 11.5% in 5 years, after moderate injury the risk was 0.7 and 1.6%, and after mild injury the risk was 0.1 and 0.6%. The incidence of seizures after mild head injuries was not significantly greater than in the general population.

Skeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy

The goals of this study were to describe the clinical course of skeletal, cardiac, and gastrointestinal muscle manifestations and trends in age at diagnosis and survival of Duchenne muscular dystrophy (DMD) patients. A retrospective cohort of 33 male patients with DMD, born between 1953 and 1983 and followed at the Mayo Clinic during their second decade of life, was studied. The mean age at DMD diagnosis was 4.6 years. Skeletal muscle weakness present in all patients at diagnosis progressed to wheelchair dependency in 32 patients (97%) by the age of 13 years (median age 10 years). Cardiac muscle failure developed in 5 patients (15%) (median age 21.5 years). Smooth muscle manifestations related to the digestive and urinary tracts occurred in 7 (21%) and 2 (6%) patients (median age 15 years), respectively. The gastrointestinal dilatations were primary in 2 patients or secondary to surgery or acute respiratory illness in 5 patients. By the end of the study period, 17 deaths had occurred (median age 17 years). Over time, there was a decrease in the time to DMD diagnosis (P = .05) but no significant change in survival (P = .44). Cardiac and smooth muscle manifestations occur late in the course of DMD. Clinical gastrointestinal symptoms related to smooth muscle function most often were secondary to surgery or a respiratory illness. In recent years, the diagnosis of DMD has been made at a younger age, but survival has not changed.

Cerebral palsy Survival rates, associated handicaps, and distribution by clinical subtype (Rochester, MN, 1950–1976)

We identified 64 cases of cerebral palsy (CP) born to Rochester residents in 1950-76. The majority (73%) had one of the spastic syndromes. Ataxic and dyskinetic syndromes constituted 16% and 6%, respectively. Survival follow-up was available through 1980 birthdates. Using life-table methods, we calculated survival rates for the first 10 years of life. For severely or profoundly retarded children, survival was 68% at 5 years and 54% at 10 years. All others survived through their 1980 birthday. Follow-up of CP resolution was available through the seventh birthday. Cases born in 1968-76 had the highest resolution rate (30%). For mild CP, the 1968-76 resolution rate was 86%.

Cisplatin-based chemotherapy in primary central nervous system germ cell tumors

We report a retrospective review of our experience with cisplatin-based chemotherapy in eight patients (ages 9–44 years) with histologically confirmed primary central nervous system germ cell tumors. Five patients received chemotherapy as the primary treatment, radiation therapy being administered either at completion of chemotherapy or between chemotherapy courses. Three patients received cisplatin-based chemotherapy for recurrent disease after prior radiation therapy and/or surgery. Four of five patients treated with chemotherapy at diagnosis are in complete remission at 11–14 months from diagnosis. The remaining patient twice achieved complete remission prior to dying of progressive disease 16 months after diagnosis. Two of three patients treated with chemotherapy for recurrent disease are in complete remission at 20 and 26 months; the remaining patient deteriorated after the first cycle of chemotherapy and expired six months thereafter. Overall, of seven patients evaluable for response, five achieved complete remission with chemotherapy alone, and two with chemotherapy and radiation therapy. Our results confirm previous reports of high complete remission rates utilizing cisplatin-based chemotherapy in conjunction with radiation therapy. Prospective evaluation of cisplatin-based chemotherapy followed by radiation therapy is warranted.

Topical Review Article: Medulloblastoma: I. Clinical, Diagnostic, and Therapeutic Overview

Medulloblastoma, the most common embryonal tumor of the central nervous system, affects both children and adults. It poses a significant therapeutic challenge in that age-dependent differences exist, not only in their pathobiology, but in the efficacy of chemotherapy and radiotherapy. This is particularly the case in very young children, whose still developing nervous system exhibits a low tolerance to radiotherapy. We review the epidemiology, clinical presentation, radiologic features, and current therapeutic concepts relative to this unique neoplasm. Efforts are made to highlight clinical controversies. (J Child Neurol 1992;7:142-155).

Juvenile Temporal Arteritis Revisited

We describe a case of arteritis involving the superficial temporal artery in an 8-year-old boy. After a 2-week prodrome of headache in the right temporal region, a painful pulsatile 6-mm nodule developed. No history of trauma or systemic disease was noted. The differential diagnosis included vasculitis or thrombosis of a vascular malformation of the temporal artery. The lesion was surgically excised for both diagnostic and cosmetic reasons. Histologic features of the nodule were diagnostic of juvenile temporal arteritis and characterized by non-giant cell granulomatous inflammation of the temporal artery, occlusive fibrous intimal proliferation, and microaneurysmal disruption of the media. At 12-month follow-up, the patient was well; no recurrent lesions or systemic disease was noted. Although rare, this disease should be recognized as arteritis that affects the external carotid circulation and should not be confused with classic giant cell temporal arteritis. If physicians are aware of this benign inflammatory disease of the temporal artery in children and young adults, unnecessary treatment will not be administered.

Hepatotoxic effects in a child receiving valproate and carnitine

L-Carnitine supplementation has been recommended to prevent the fatal hepatotoxic effects associated with valproic acid. We report on a child with fatal valproate-related hepatotoxic effects despite this supplementation.

Cerebral palsy—trends in incidence and changes in concurrent neonatal mortality Rochester, MN, 1950‐1976

We studied all identified cases of cerebral palsy (CP) born to residents of Rochester, MN, during a 27-year period. Incidence rates per 1,000 neonatal survivors were computed for three 9-year intervals. For children with persisting findings, the incidence rate of all syndromes declined from 2.3 to 1.6. For spastic syndromes, this trend was more marked (2.1 to 0.9). For newborns with birthweight over 2,500 g, both CP incidence and neonatal mortality rates (NMR) declined in parallel. For the low birthweight neonate, coincident with a precipitous drop in NMR, the CP incidence rate remained essentially unchanged. In the face of increasing neonatal survival, the overall incidence rate of CP declined. The decrease in the absolute risk of CP was limited to the > 2,500-g neonatal survivor.

Seizures Presenting as Episodic Hypersomnolence

Important distinctions must be drawn between disorders of sleep and epilepsy. Epileptic seizures, frequently with complex symptoms, occur during sleep; on the other hand, certain sleep disorders, such as narcolepsy, somnambulism, and night terrors, may resemble complex partial seizures (CPS) (Peled and Lavie, 1986; Culebras and Magaiia, 1987; Kales et al., 1987; Donat and Wright, 1989; Shouse, 1989; Mahowald and Ettinger, 1990; Mitler et al., 1990). We document the unusual case of a patient who had CPS alternating with sleep and presenting as periods of hypersomnolence.

Preliminary communication--treatment of primary brain tumors recurrent after irradiation with aziridinylbenzoquinone (AZQ;NSC-182986).

Twenty-nine patients with primary brain tumors recurrent or progressive after cerebral irradiation were treated with AZQ. Twenty of the 29 patients had also failed prior chemotherapy. CT scan-documented tumor regressions were noted in 17.2% (5/29) and ranged from 15.0% (3/20) in patients with prior chemotherapy to 22.2 (2/9) in patients without prior chemotherapy. Myelosuppression was the only significant toxicity noted. AZQ is worthy of further studies in patients with primary brain tumors.