Ian J. Hopkins

Acute transverse myelopathy in childhood

The clinical features and outcome of idiopathic acute transverse myelopathy were reviewed for 21 children aged between seven months and 14 years. Pain, most commonly in the back, was the initial symptom for 12 patients; for another six it was weakness and for two urinary retention. All patients had weakness of the legs, and 11 had arm weakness as well. Five patients had very acute onset of severe weakness and were unable to walk within three hours of onset of symptoms. Two children made no significant recovery; for the remainder onset of recovery was evident within two to 17 days. 12 patients later were normal or had only minimal neurological deficit, but nine had a poor outcome with major disturbance of motor or sphincter function. Only one of the five with very acute onset had a good outcome. Over–all, the prognosis after acute transverse myelopathy in childhood is a little better than that reported for adults.

Resective surgery in infants and young children with intractable epilepsy

Despite the relatively high incidence of epilepsy during the first few years of life, and its documented adverse impact on development, few studies have documented the long term outcomes following epilepsy surgery in infants and young children. We report a consecutive series of 11 patients under 3 years of age who underwent excisional surgery for intractable seizures. A total of 16 resective procedures were performed, and comprised functional hemispherectomy, temporal lobectomy, and lesionectomy. The mean follow-up period was 3 years. At follow-up, 73% (8/11) were seizure-free, with over half of these patients able to cease anti-epileptic medications. Acceleration of neurodevelopment was seen in all patients in whom preoperative developmental delay was present. Quality of life was enhanced in all patients. This series confirms that excellent results can be obtained following excisional surgery in well-selected infants and young children with intractable seizures.

Landau-Kleffner syndrome: Six patients including discordant monozygotic twins

This is the first published report of Landau-Kleffner syndrome occurring discordantly in monozygotic twins. The implications of this finding in the understanding of the etiology of this condition are discussed. Five other patients are reported and comparisons are drawn with other series.

Occlusive disease of the vertebro-basilar arterial system in childhood.

Three examples of vertebro‐basilar occlusive disease in children, documented by arteriography, are described. One patient had recurrent episodes of vertigo for several months, and later dysarthria, ataxia and a hemiparesis together with fluctuating ocular palsies. Arteriography showed widespread changes in the vertebro‐basilar system similar to those of so‐called ‘arteritis’. A second case had similar clinical manifestations but arteriography showed complete occlusion of one vertebral artery. The cause of the vascular occlusion was not established in these cases. The third case developed sudden coma in the course of bacterial endocarditis and arteriography showed a narrow attenuated basilar artery presumably due to embolism.

Idiopathic Hypothalamic Dysfunction With Dilated Unresponsive Pupils: Report of Two Cases

Idiopathic hypothalamic dysfunction is a rare but well-defined entity in childhood characterized by adipsia-hypernatremia, obesity, poor thermoregulation, and disturbance of pituitary function. Two cases of idiopathic hypothalamic dysfunction are described. There are 10 previously reported cases in the literature, and the clinical features are compared. The present cases are unique in that the patients also had bilaterally dilated unresponsive pupils. In the first case, there was no demonstrable pathology at autopsy; in the second case, lymphocytic infiltration of the hypothalamus and midbrain associated with neuronal loss was present at autopsy. Possible etiologies are discussed. (J Child Neurol 1994;9:320-325).

Acute Respiratory Failure Precipitated by General Anesthesia in Leigh's Syndrome

Three patients with Leighs syndrome developed respiratory failure following general anesthesia. Although all three had respiratory symptoms prior to the anesthetic, the diagnosis was not suspected at the time of the procedure in two of the children. We reviewed the case notes of 16 other patients with Leighs syndrome. Eight had received anesthetic agents without incident. Although the majority subsequently developed respiratory abnormalities and died with respiratory failure, this problem was not evident at the time of anesthesia. In the presence of respiratory abnormalities, general anesthesia carries significant risks in Leighs syndrome. (J Child Neurol 1990;5:137-141).

Arteriographic Demonstration of Vascular Lesions in the Study of Neurologic Deficit in Advanced Haemophilus Influenzae Meningitis

Five patients are reported with severe neurological sequelae following haemophilus influenzae meningitis. Cerebral angiography showed narrowing or occlusion of the terminal carotid arteries or their branches in all cases. Venous sinus occlusion, and prominent vasodilatation of capillaries in the region of the middle cerebral artery were also present in one patient, in whom clinical features were consistent with cerebral necrosis in this region.

Acute Pseudobulbar Palsy Due to Bilateral Focal Cortical Damage: The Opercular Syndrome of Foix-Chavany- Marie

Two children are described who suddenly developed an encephalitic illness with intractable bilateral facial seizures. The seizures subsided over several days, but the children were left with the signs of pseudobulbar palsy and are unable to speak or swallow effectively. Bilateral destructive lesions in the opercular regions evolved on computed tomographic scans. Both children were treated with acyclovir relatively early in the illness, and cerebrospinal fluid and serum antibodies support the diagnosis of herpes simplex virus encephalitis. (J Child Neurol 1989;4.-131-136).

Can Head Injury Influence the Site of Demyelination in Adrenoleuko dystrophy

Adrenoleukodystrophy is an inherited disorder, in which there is degeneration of white matter of the central nervous system. The disorder presents classically in males during the first decade, and although early clinical features may be asymmetrical, radiological and pathological features are essentially generalised. Two boys are reported with proven adrenoleukodystrophy, who at first had focal clinical and radiological features, with the foci in the region of local trauma. This raises the possibility that a local insult may have precipitated or hastened the degenerative process.

A Case of Krabbe's Leukodystrophy Without Globoid Cells

&NA; Krabbes globoid cell leukodystrophy is a rare hereditary progressive neurological disease of infants, in which there is deficient activity of galactosylceramide β‐galactosidase. The pathological hallmark is the presence of multinucleated globoid cells in the white matter associated with severe myelin depletion and gliosis.3,4,5 We report a second case where galactosylceramide β‐galactosidase deficiency was proven but no globoid cells were found in the brain. Symptoms began within the first 10 months of life and a deficiency of galactosylceramide β‐galactosidase activity was demonstrated in peripheral blood leukocytes and skin fibroblasts. The child survived till 8 yrs 7 mths. The reason for the absence of globoid cells is not clear but may be related to different effects of the gene mutation on the four substrates or possibly the interaction of sphingolipid activator protein‐2.23