Arthur L. Prensky

PARTIAL CHARACTERIZATION OF A NEW MYELIN PROTEIN COMPONENT

A new protein component has been demonstrated in myelin isolated from rat whole brain and from white matter dissected from bovine, dog and rabbit brain. It is also present in rabbit optic nerve. It does not appear to be present in other subcellular fractions of rat brain. It has a molecular weight of 20,540 ± 490(S.D.), as determined by polyacrylamide gel electrophoresis in sodium dodecyl sulphate. A rapid procedure for the isolation of myelin is also described.

Diagnosis and treatment of migraine in children

We here enumerate criteria that we believe are suitable for the diagnosis of migraine in children. Using these criteria, we identified 84 children retrospectively, and studied their illness for 3 to 9 years thereafter. The majority were male, and 47 patients had frontal headaches. EEGs were performed in 64 children: 17 were paroxysmal, but 7 patients never developed seizures. Referral to a neurologist occurred when there was a marked increase in the frequency or severity of headaches. Irrespective of the form of treatment, about one-half of all patients had more than a 50 percent reduction in headache frequency in the 6 months following their initial visit to a neurologist.

A progressive neurologic syndrome in children with chronic liver disease.

A progressive neurologic syndrome developed in six children with longstanding cholestatic liver disease. The neurologic abnormalities included areflexia, gait disturbance, decreased proprioceptive and vibratory sensation, and paresis of gaze. Serum vitamin E concentrations were uniformly low. Neuropathological studies carried out in two of the three fatal cases revealed degeneration of the posterior column, selective loss of large-caliber, myelinated axons in peripheral nerve, and spheroids in the gracile and cuneate nuclei. These lesions are similar to those found in animals with experimentally induced vitamin E deficiency. We therefore speculate that the neurologic syndrome in these children may be the result of chronic vitamin E malabsorption.

Migraine and migrainous variants in pediatric patients.

Children suffer from migraine and migraine variants which occasionally present problems with regard to diagnosis, often being mistaken for epilepsy or structural brain disease. Criteria for classification and diagnosis are presented, methods of differential diagnosis are proposed, and two possible approaches to treatment are suggested.

Clinical and electroencephalographic manifestations of vascular lesions of the pons

THE INFLUENCE EXERTED by specific brainstem structures upon the state of alertness and patterns of electrocortical activity in man remains poorly defined, despite the continuing interest this problem has held for neurologists. Much of our information derives from the results of skillfully placed lesions in the brainstem of the cat. Such studies suggest that two functionally antagonistic and anatomically separate pathways within the brainstem reticular formation play a crucial role in modulating both the electrical organization and reactivity of the cerebral cortex and the state of alertness of the animal.1-13 In man, only the analysis of welldefined, stable lesions resulting from vascular disease presents a situation which approaches that found in the experimental animal. The reported incidence of EEG abnormalities associated with vascular lesions of the brainstem varies widely but averages about 30% in most large series,14-lQ although in some, as many as 70% of the tracings have been considered abnormal.20921 The EEG changes are usually slight, consisting of diffuse or bilateral slowing, especially in the temporal regions.16-1**20.21 Paroxysmal discharges and sharp activity, also most conspicuous in the temporal leads;lss20-22 focal abnormalities;14 low-voltage fast records;29-25 and generally low-voltage records have also been observed.1BJss20 In the few patients in whom such data have been recorded, the effect of photic stimulation has usually been regarded as normal,14 while sleep studies have been reported as normal or lacking rapid-eye-movement (REM) patterns.14~~6 In general, normal EEG configurations have been considered compatible with small lesions, laterally and caudally situated in the brainstem, while large, medial and rostral infarcts have been thought to be associated with a relatively high incidence of EEG abnormalities.lO.22.27 Unfortunately, such conclusions are based largely upon case studies lacking necropsy verification. There have been few attempts to correlate behavioral and EEG data with the site of brainstem injury. A review of the literature has yielded only 12 cases, with adequate behavioral and EEG studies, in which there was a major lesion in the pons or lower midbrain without significant abnormality in the cerebral hemispheres or rostral midbrain.21.*3,24,2-2 Analysis of this material together with similar data from 8 additional cases form the basis of the present report (see table). Incomplete accounts of 2 of these cases have been previously published.33.34 EEGs were obtained on Grass Model 4 or 6 machines using either 8 or 16 channels and the International 10-20 system of electrode placement. Bipolar and unipolar montages were used in all cases; in the latter instance, a balanced neck-chest lead served as the common reference.

Steroid‐responsive encephalomyelitis in childhood

The syndrome of parainfectious encephalomyelitis evolves from an antecedent infection. Several etiologic agents have been associated with this complication, although the pathogenesis in each instance may prove to be more uniform. Considerable evidence suggests that the syndrome is mediated immunologically. The seven cases reported here were clinically similar, although the infectious etiologies were diverse. Leptospirosis antedated the neurologic syndrome in two cases, and a “viral” illness preceded the other five cases. The evolution of the syndrome was slowly progressive in each case, and six patients had prominent involvement of rhombencephalic structures. The progressive course was reversed rapidly with eventual full recovery in each instance after initiation of corticosteroid therapy. Our experience with these cases coupled with a review of the literature suggests that corticosteroid therapy should be considered in the subacute or chronic cases of parainfectious encephalomyelitis.

The effect of undernutrition on the development of myelin in the rat central nervous system.

Newborn rats were segregated into litters of 6 and 22 pups. Undernourished groups were maintained for 21 and 53 days at which time a portion of each group was sacrificed and the remainder refed until 121 days. The amount of myelin isolated from the central nervous system was decreased in nutritionally deprived animals but there was little change in its chemical composition or intereference in the normal chemical maturation of the membrane. The only consistent change was a reduction in phosphatidyl ethanolamine plasmalogen. This deficit tended to be corrected by nutritional rehabilitation and was most complete in those animals refed ad lib. diets for the longest time periods prior to sacrifice. The relatively normal chemical composition of myelin obtained from undernourished animals differs from changes described in myelin membranes isolated from patients with destructive or degenerative diseases of the nervous system. This seems to be further evidence that the reduction in myelination in undernutrition is a result of decreased synthesis of the membrane.

Acquired epileptiform opercular syndrome: a second case report, review of the literature, and comparison to the Landau-Kleffner syndrome.

Summary: A 5‐year‐old girl developed recurrent prolonged episodes of severe oral apraxia, dysarthria, and drooling, similar to the opercular syndrome in children. Each episode lasted several weeks to >6 months and was associated with exacerbation of epileptiform activity in her EEG. Electrographic status epilepticus during slow wave sleep (ESES) was recorded during three of the exacerbations. The EEG improved markedly when clinical symptoms subsided. Antiepileptic drugs (AEDs) were not effective, although there was some improvement when they were combined with a ketogenic diet. A similar case was described by Roulet et al. We believe that this is a distinct epileptic syndrome, equivalent to the Landau‐Kleffner syndrome (LKS).

The moyamoya syndrome associated with irradiation of an optic glioma in children: Report of two cases and review of the literature

We report two cases of the moyamoya syndrome which became clinically apparent after irradiation of an optic glioma during childhood. A summary of 14 cases of this syndrome following irradiation of intracranial tumors is also presented. Nine of these cases were optic gliomas; five were found in children with neurofibromatosis, another disorder that has a strong association with the moyamoya syndrome. The effectiveness of irradiation of optic gliomas in childhood is not definitely established. The possibility of inducing serious vascular disease is a further reason for caution when considering irradiating these tumors.

Management of Seizure Disorders: Selected Aspects. Part II.

This article discusses the management of seizure disorders, stressing selected aspects including (1) the pharmacology of drugs used in the control of seizures, especially the usefulness of measuring concentrations of anticonvulsants in blood in guiding therapy; (2) the treatment of prolonged seizures, particularly status epilepticus, in infants and children; (3) the use of the ketogenic diet as an adjunct in the therapy of epilepsy; and (4) the place of surgical treatment in the control of seizures resistant to medical management.