Francis S. Wright

Cerebral dysfunction after chronic hypoxia in children

Although the long-term effects of acute anoxia have been studied, the effects of chronic hypoxia on the developing human brain have received little attention. We studied children with a cyanotic congenital heart defect to assess the impact of chronic hypoxia by eight measures: neurologic examination, visual evoked response, EEG, behavioral adjustment, cognitive, perceptual-motor, and attentional functioning, and school performance. On outcome evaluation, these children evidenced diverse neurophysiologic dysfunction. Chronic hypoxia was associated with impaired motor function, inability to sustain attention, and low academic achievement.

Simultaneous Infantile Spasms and Partial Seizures

We report 11 infants with infantile spasms who had partial seizures that occurred concurrently with the infantile spasms. We studied this phenomenon with time-locked video electroencephalography. The partial seizures began before the infantile spasms and continued after one or more infantile spasms in seven patients. An infantile spasm occurred at the beginning of the partial seizure in five patients. (One patient had both seizure types simultaneously.) The clinical descriptions of these combinations of seizures were confusing, since they did not match known seizure types. The genesis of these seizures may involve an interaction between processes of focal cortical epileptogenesis (partial seizures) and brainstem synchronization (infantile spasms) (J Child Neurol 1991;6:246-250).

Seizures in series : similarities between seizures of the west and Lennox-Gastaut syndromes

Summary: We observed seizures resembling infantile spasms in patients with Lennox‐Gastaut syndrome (LGS). Infantile spasms, the type of seizures that occurs in patients who have West syndrome, have been well characterized by video‐EEG studies and typically occur as a series of sudden generalized flexor or extensor jerks. The seizure types that occur in LGS have not been as clearly delineated. Some patients with West syndrome (WS) in early infancy later develop LGS. Using intensive video‐EEG monitoring, we evaluated 14 LGS patients who had seizures that occurred in series. Clinically the seizures greatly resembled infantile spasms, and the ictal EEG changes were identical to those that occur with infantile spasms. These findings expand the number of features known to be shared by these two syndromes and strengthen the hypothesis that the two syndromes represent age‐related manifestations of similar epileptogenic processes.

Episodic symptoms mistaken for seizures in the neurologically impaired child

We found neurologically impaired children studied by time-locked video-EEG to have episodes of abnormal behaviors which had been mistaken for epileptic seizures. Recognition that other neurologically abnormal phenomena can closely mimic epilepsy is important for prevention of erroneous diagnoses of epilepsy, and thus overtreatment, in this patient population.

Acute Chemical Pancreatitis Associated with Carbamazepine Intoxication

Summary: A 5‐year‐old mentally retarded child developed laboratory evidence of pancreatitis during accidental acute carbamazepine (CBZ) intoxication. He had been seizure‐free with CBZ for 4 years for a seizure disorder with no obvious toxicity. CBZ had been discontinued 5 months before he was admitted to the hospital. After he accidentally ingested a CBZ overdose, he was found vomiting and lethargic. Serum amylase and lipase levels were increased for several days. With supportive treatment and no CBZ, he recovered and serum amylase and lipase levels returned to normal. No other causes of pancreatitis were identified. Therefore, most likely the chemical pancreatitis was associated with the acute CBZ intoxication.

Familial Adrenal Insufficiency, Achalasia, Alacrima, Peripheral Neuropathy, Microcephaly, Normal Plasma Very Long Chain Fatty Acids, and Normal Muscle Mitochondrial Respiratory Chain Enzymes

Adrenal insufficiency has been associated with adrenoleukodystrophy and adrenomyeloneuropathy. In these diseases, plasma very long chain fatty acids are elevated. Peripheral neuropathy is frequently seen in adults with adrenomyeloneuropathy. We encountered two first cousins with adrenal insufficiency, who also developed peripheral neuropathy, achalasia, alacrima, and microcephaly. However, plasma very long chain fatty acids, pipecolic acid, phytanic acid, and cranial computed tomographic scan were normal. Muscle mitochondrial respiratory chain enzymes were also normal. This syndrome of adrenal insufficiency, achalasia, alacrima, microcephaly, and peripheral neuropathy is different from either adrenomyeloneuropathy or adrenoleukodystrophy. (J Child Neurol 1994;9:135-138).

THE EFFECTS OF THE FALLING THERAPEUTIC AND SUBTHERAPEUTIC BARBITURATE BLOOD LEVELS ON ELECTROCEREBRAL SILENCE IN CLINICALLY BRAIN-DEAD CHILDREN

In a retrospective study at a large childrens hospital, we identified 92 children who had received barbiturates that were simultaneously discontinued at the time they were being evaluated for brain death in the presence of electrocerebral silence and clinical brain death by physical exam. Of these 92 children, 67 had barbiturate levels that were monitored from initial therapeutic or subtherapeutic levels. Repeat EEGs were obtained in 76 patients, and in all electrocerebral silence and clinical brain death (by exam) persisted despite the lower barbiturate levels. The study suggests that therapeutic and subtherapeutic barbiturate levels have no effect on the outcome of children who fulfill the criteria for brain death.

Review Article: Sleep, Epilepsy, and the EEG in Infancy and Childhood

Each of the major epileptic syndromes that occur in infants and children demonstrates relationships to sleep and wakefulness that are particular to that syndrome. These relationships include activation or suppression of clinical seizures during certain portions of the sleep-wake cycle, differences in symptomatology of the seizures or in seizure type, alterations in distribution or morphology of epileptiform waveforms, and changes in duration and composition of sleep stages. Knowledge of the interactions between sleep and seizures helps to increase understanding of the physiological mechanisms underlying epilepsy, as well as to improve clinical diagnosis. (J Child Neurol 1989;4:84-94).

Dietary essential fatty acids, vitamin E, and Charcot‐Marie‐Tooth disease

Twenty patients with type I Charcot-Marie-Tooth disease received dietary supplementation with the essential fatty acids (EFA), linoleic and gamma-linolenic acids, and vitamin E. A 3-month blinded trial of placebo (paraffin oil and vitamin E, 81.6 IU/d) was followed by 1 year of 3 grams daily of EFA and vitamin E. Serum fatty acid values doubled, but total esterified fatty acid proportions did not change. Arachidonic acid proportions correlated with the amount of prostaglandin-mediated lymphocyte suppression measured at the same times. Improvement demonstrated at the end of the placebo period by neuropsychological tests and neurologic examination was maintained during the 1 year of EFA supplementation. This effect may reflect a membrane stabilization benefit of vitamin E.

Clinical Imitators of Infantile Spasms

We report 53 infants who by clinical history were thought to have infantile spasms but who video-electroencephalograms showed were having other episodes that closely mimicked infantile spasms. Nine patients had other types of seizures. Forty-five patients had episodic symptoms that were not seizures: 11 patients had spasticity, four had gastroesophageal reflux, and the other patients had nonepileptic myoclonus, including 19 patients with benign neonatal sleep myoclonus. Three patients had more than one type of symptom. Infantile spasms imitators occurred in neurologically normal or abnormal infants, in patients with normal or abnormal interictal electroencephalograms, and in patients who also had previous or current infantile spasms. Differentiation of these episodes from infantile spasms prevented the initiation or continuation of anticonvulsant treatment appropriate for infantile spasms but inappropriate for these other behaviors. (J Child Neurol 1992;7:395-399).