Prevalence of white matter lesions and stroke in children with migraine.

Objectives: To determine the prevalence of white matter lesions (WMLs) and infarcts in children with migraine and whether pediatric migraine could be a risk factor for silent ischemic lesions or stroke.

Methods: Prospectively collected data from 1,008 pediatric patients with headache were reviewed. The MRI data were collected and retrospectively reviewed.

Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.

Objective: The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence. For this parameter, the authors reviewed available evidence on the evaluation of the child with recurrent headaches and made recommendations based on this evidence.

Methods: Relevant literature was reviewed, abstracted, and classified.

Childhood Migraine Headache Syndromes.

The treatment of migraine headache in children depends on the following: a) defining the underlying cause; b) the frequency of the attacks; and c) the severity of the disability produced by the pain. Any medication taken to relieve pain is most effective if taken at maximum dose at the onset of the headache. The dose should be the maximum recommended by weight or age.

Progressive infantile axonal polyneuropathy.

Polyneuropathies are relatively uncommon in early infancy and the majority of affected children are found to have hypomyelinating neuropathies. Axonal sensorimotor neuropathies have been described in childhood but the majority of affected children present at or after 6 months of age, have nonprogressive courses, and achieve the ability to walk, albeit late. Here we present three infants with infantile progressive axonal polyneuropathy from two families with nonconsanguineous parents.

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

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.

Course and outcome of acute cerebellar ataxia.

We report a study of 73 consecutive children with acute cerebellar ataxia, representing all of the children evaluated at St. Louis Children's Hospital during a 23-year-period to whom this diagnosis could appropriately be assigned. Twenty-six percent had chickenpox, 52% had other illnesses that were presumed to be viral, and in 3% the ataxia was related to immunization.

Neurodegenerative diseases of childhood: MR and CT evaluation.

To evaluate the diagnostic utility of MR and CT in neurodegenerative diseases of childhood, we examined 63 children (MR in 44, CT in 53) carrying diagnoses of Leigh disease (14 patients), various metabolic diseases (13 patients), leukodystrophies (13 patients), other specific degenerative diseases (10 patients), and unclassified neurodegenerative disorders (13 children). Magnetic resonance yielded positive findings in 86% and CT in 81%. Lesion extent and conspicuity were consistently greater with MR.

Leigh disease (subacute necrotizing encephalomyelopathy): CT and MR in five cases.

Magnetic resonance (MR) imaging and CT were used in supporting the diagnosis of Leigh disease (subacute necrotizing encephalomyelopathy) in five patients. Two cases were confirmed at postmortem examination, and three patients have laboratory and/or clinical histories suggestive of Leigh disease. Four cases demonstrated findings in the basal ganglia, especially the putamina, with symmetrical areas of low attenuation on CT.

Computed tomography and magnetic resonance imaging in children with rare movement disorders.

Both CT and MR can aid in the differentiation between benign and less favorable pediatric movement disorders. However, MR is the more sensitive imaging modality and thus preferable. This was demonstrated in 18 patients with conditions ranging from idiopathic dystonias to aminoacidopathies, Leigh's disease, and Hallervorden-Spatz disease.

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 steroid treatment of hereditary motor and sensory neuropathy.

A placebo or methylprednisolone (45-60 mg/M2) was administered in a crossover study as a single morning dose on alternate days to fourteen patients who had a familial progressive polyneuropathy that either began or was maximum in the distribution of the peroneal nerves. Neither the patients nor the examining physician were told whether the patient was taking a placebo or steroid but the steroid side-effects made a double-blind trial impossible. Patients were evaluated at the initiation of the study, the time of crossover, and at the conclusion of the study.

Intelligence in epilepsy: a prospective study in children.

A prospective study tested the stability of the IQ in children with seizure disorders. Seventy-two children with epilepsy underwent psychological evaluations within two weeks of initial diagnosis and yearly thereafter for an average of 4 years. Forty-five of the patients also had a nonepileptic sib evaluated in parallel.

Is the academic pediatric neurologist an endangered species?

Some argue that the physician-researcher is an endangered specie and that the 'bedside connection' is severely strained. Our survey of recent graduates of pediatric neurology training programs does not substantiate this. Almost one-half of graduating pediatric neurology residents are embarking on full-time, university-based careers.

An infant with chronic, relapsing polyneuropathy responsive to steroids.

A seven-week-old infant presented with an acute flaccid paraparesis. Her subsequent course was punctuated by numerous exacerbations, in association with minor intercurrent illness and remissions produced by corticosteroid treatment. Postmortem examination revealed a chronic inflammatory polyradiculopathy.

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.

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.