Pulsatile tinnitus in a 16-year-old patient.

This report describes a child presenting with pulsatile tinnitus, most likely resulting from increased intracranial hypertension secondary to cerebral venous thrombosis. He was found to be homozygous for a prothrombin gene (G20210A) mutation as well as heterozygous for a factor V Leiden mutation. Physicians need to pursue and determine the cause of pulsatile tinnitus and intracranial hypertension.

Cyclic vertigo with predictable recurrence.

The long-term follow-up of six children with "cyclic vertigo," that has recurred for years, at strikingly predictable intervals, is presented. Vertigo and photophobia, lasting several hours, start early in the morning, daily for several consecutive days, and recur at predictable intervals without evidence of long-term neurological deficits. The interval between events increases with age.

A simplified diagnostic approach to dizziness in children.

The objective of the study was to validate the effectiveness of a questionnaire and computer-assisted algorithm in diagnosing children with dizziness or vertigo. Dizziness and vertigo are common complaints in children, causing an extensive, often unnecessary evaluation. A pediatric "dizziness questionnaire" was designed and a computer-assisted algorithm was developed to facilitate the diagnostic task.

Acute onset of infantile spinal muscular atrophy.

Two patients with acute generalized weakness and areflexia are presented. The electrophysiologic studies in both revealed evidence of decreased conduction velocity and mixed axonal and demyelinating neuropathy, suggestive of the diagnosis of Guillain-Barré syndrome. The young ages of the patients and their failure to respond to immunoglobulin therapy were the major clues to the final diagnosis of spinal muscular atrophy type I.

Acute necrotizing encephalopathy presenting as a basal ganglia syndrome.

Acute necrotizing encephalopathy is a relatively new disease. The characteristic clinical findings are of febrile illness followed by rapid deterioration in mental status and seizures. The hallmark of the disease is multifocal bilateral symmetric lesions affecting the thalamus, hypothalamus, brainstem tegmentum, cerebral white matter, and cerebellum.

Biphasic course of infant botulism.

The syndrome of infant botulism, characterized by constipation, poor feeding, hypotonia, poor head control, and bulbar involvement, is typically a monophasic disease. We describe a 7-month-old infant with a recurrence of illness 13 days after resolution of the presenting signs. The source of infection was unknown and the only potential risk factors were exclusive breastfeeding and decreased bowel movements, which by themselves cannot explain the recurrence.

Neurologic manifestations of Mycoplasma pneumoniae infections: diverse spectrum of diseases. A report of six cases and review of the literature.

Mycoplasma pneumoniae is a common cause of upper and lower respiratory tract infections of varying severity. It is also responsible for producing a wide spectrum of nonpulmonary manifestations including neurologic, hepatic, cardiac, and hematologic diseases. The neurologic manifestations are reported to be the most common nonpulmonary manifestations.

Coma as an acute presentation of adrenoleukodystrophy.

X-linked adrenoleukodystrophy is a metabolic disorder with broad clinical variations. A 4-year-old male admitted to the hospital with fever, hypotension, and coma as the presenting signs of adrenoleukodystrophy is reported. The initial presentation followed by rapidly developing disseminated intravascular coagulopathy and multiorgan failure suggested an initial diagnosis of septic shock.

The value of early postictal EEG in children with complex febrile seizures.

Purpose: To assess the usefulness of an early postictal EEG in neurologically normal children with complex febrile seizures.

Methods: We conducted a retrospective chart review of all neurologically normal children who were hospitalized over a period of 2.5 years after complex febrile seizures, and had an EEG up to 1 week after the seizure.

Risk of excessive weight gain in epileptic children treated with valproate.

We sought to identify factors associated with excessive weight gain in children treated with valproate, excluding patients fed by gastrostomy or treated with medications known to affect appetite (eg, stimulants). Weight and height were recorded before treatment and at the time of follow-up; a measure of adiposity, body mass index, was computed and expressed in kg/m2, and weight and height for age were converted to Z-score. Putative risk factors included sex, age at start of treatment, monotherapy at start of treatment, duration of follow-up, mental retardation, seizure type (generalized or partial), etiology (idiopathic or cryptogenic versus remote symptomatic), and dose of valproate.

Subdural empyema in 12-year-old girl: the value of magnetic resonance imaging.

A 12-year-old girl presented with an acute history of fever, headaches, and focal neurological deficits. An initial computed tomography (CT) scan of the head was nondiagnostic whereas plain and gadolinium-enhanced magnetic resonance imaging (MRI) detected an extensive subdural empyema. The report emphasizes the hazard of relying on a nondiagnostic CT scan in a septic patient with deteriorating neurological function, and the need of MRI with contrast enhancement if subdural empyema is a serious clinical concern.

The value of brain imaging in children with headaches.

Objective: To determine the value of performing computed tomography (CT) on magnetic resonance imaging (MRI) studies in children with chronic headaches.

Background: Headache is a common complaint in children. With the proliferation of brain imaging centers and the increasing patient demand for CT or MRI studies, brain imaging has become widely used to evaluate headaches.

Prognosis and treatment of seizures in children with acute lymphoblastic leukemia.

We reviewed the records of 127 consecutive pediatric patients with acute lymphoblastic leukemia (ALL) to determine the incidence, timing, etiologies, and recurrence rate of seizures in this population. Patients with ALL and seizures were identified retrospectively by review of the records of all pediatric ALL patients who were diagnosed and treated during the years 1983 through March 1993 in a large tertiary-care hospital. Seventeen patients (13%) developed one or more seizures.

Dizziness in children.

The differential diagnosis of dizziness in children is extensive. It requires a careful evaluation of the patient's complaint and a systematic review of systems. Because there are endless etiologic possibilities, an algorithmic approach, taking into account the patient's complaint, age, and clinical laboratory findings, may help the clinician reach an accurate diagnosis.

New subform of the late infantile form of neuronal ceroid lipofuscinosis.

Clinicopathological studies of a series of nine children with a new subform of Jansky-Bielschowsky disease or late infantile neuronal ceroid lipofuscinosis (LINCL) is presented. The onset of this subform is between 2.5-3.

MRI in neonatal dural sinus thrombosis.

Dural sinus thrombosis in the newborn period is a rare but underrecognized condition which may cause seizures, macrocephaly, lethargy, and respiratory depression. A 10-day-old term infant with no pre- or perinatal risk factors for thrombosis presented with seizures and was found to have dural sinus thrombosis on computed tomography and magnetic resonance imaging (MRI). One week later, MRI revealed partial resolution and 3 weeks later disclosed a complete resolution of the thrombosis.

Prenatal diagnosis of intracranial teratoma. Prolonged survival after resection of a malignant teratoma diagnosed prenatally by ultrasound: a case report and literature review.

We are reporting a case of an infant with an intracranial malignant teratoma which was diagnosed prenatally by ultrasound at 37 weeks of gestational age. After a cesarean delivery, the resection of the tumor was performed at 24 h of age. This infant is currently the oldest reported survivor that carries this prenatal diagnosis.

Duchenne muscular dystrophy in a girl identified by dystrophin deficiency.

We report an isolated case of a girl aged three years six months with Duchenne muscular dystrophy. Analysis of the patient's DNA with a probe covering the DNA gene revealed no deletion. Dystrophin, studied in biopsied muscle from the patient, using antidystrophin antibody in combination with immunofluorescence, was nearly completely absent.

Kearns-Sayre syndrome presenting as renal tubular acidosis.

Renal tubular acidosis and tetany were the 1st manifestations of Kearns-Sayre syndrome in a 5-year-old child. Subsequently, he developed progressive external ophthalmoplegia, ptosis, retinopathy, heart block, and endocrinopathy. There was a 7.

Use of demand pacemaker in children with Guillain-Barré syndrome and cardiac arrhythmias.

We report 2 patients with Guillain-Barré syndrome who exhibited autonomic dysfunction, including lability of blood pressure and heart rate, frequent episodes of profound bradycardia, and occasional asystole. Both patients required insertion of a temporary venous pacemaker which was activated a few times during the following days. The potential need for ventilatory support in patients with Guillain-Barré syndrome is well known; however, death in these patients due to acute cardiovascular failure during autonomic dysfunction continues to occur.

Farber lipogranulomatosis: an unusual presentation in a black child.

Farber disease, a rare, inherited condition of lipid metabolism usually appears within the first two months of life. The patients may die in the first few years of life or may live into the second decade. We believe this patient to be the first black American reported with Farber disease.

Post-traumatic vertigo in children: a diagnostic approach.

The relatively high incidence of persistent post-traumatic headache and vertigo in children and adolescents presents a diagnostic and therapeutic challenge. It is often difficult to differentiate between functional complaints generated by psychological trauma or compensation-seeking and symptoms reflecting an organic etiology. The clinical and laboratory findings of 22 patients with post-traumatic headaches and vertigo were delineated into five major diagnostic categories: labyrinthine concussion, whiplash syndrome, basilar artery migraine, vertiginous seizures, and a non-specific post-traumatic dizziness.