Lydia Eviatar

The Value of Early Postictal EEG in Children with Complex Febrile Seizures

Summary: Purpose: To assess the usefulness of an early post‐ictal EEG in neurologically normal children with complex febrile seizures.

Neurologic Manifestations of Mycop lasma 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. We describe six patients demonstrating the protean neurologic manifestations of Mycoplasma pneumoniae infections. Four patients presented with the central nervous system manifestations of pyramidal and extrapyramidal tract dysfunction, seizures, cognitive abnormalities, and cerebellar dysfunction. Two patients presented with transverse myelitis. The outcome of this condition ranges from normal to severe residual deficits. Increased awareness of this disease entity may facilitate early diagnosis and thereby expedite starting appropriate therapy that may modify the outcome.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. We describe six patients demonstrating the protean neurologic manifestations of Mycoplasma pneumoniae infections. Four patients presented with the central nervous system manifestations of pyramidal and extrapyramidal tract dysfunction, seizures, cognitive abnormalities, and cerebellar dysfunction. Two patients presented with transverse myelitis. The outcome of this condition ranges from normal to severe residual deficits. Increased awareness of this disease entity may facilitate early diagnosis and thereby expedite starting appropriate therapy that may modify the outcome.

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. Patients with post-traumatic hearing loss were excluded from this study because they represent a group with different diagnostic problems and more recognizable organic pathology. Each patient had a complete neurologic evaluation including specific clinical vestibular tests (i.e., stepping test, reinforced Romberg, past-pointing evaluation, and positional tests using the Nylen-Hallpike maneuver. Laboratory studies included skull x-ray, computed tomography, electroencephalography, electronystagmography, and audiologic assessment. Symptoms, signs, and tests were evaluated in each category of post-traumatic vertigo to help establish the diagnosis and initiate treatment.

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. Clinicians should consider the diagnosis of neonatal dural sinus thrombosis in infants presenting with seizures and/or increased intracranial pressure even in the absence of risk factors or when the cranial computed tomography is normal. MRI is the most sensitive diagnostic tool to establish the diagnosis and permit a noninvasive follow-up, contributing to our understanding of the natural history, associated pathology, and prognosis of this condition.

Aminoglycoside ototoxicity in the neonatal period: possible etiologic factor delayed postural control.

Forty-three infants, treated with aminoglycosides during the neonatal period, were tested repeatedly over a period of two to five years for neurovestibular responses. Results of testing were compared with those obtained from a group of 276 healthy newborns followed simultaneously. No abnormalities were found in the untreated group. Among the treated infants, three had a sensorineural hearing loss and eight had laboratory evidence of vestibular dysfunction and delay of head and postural control.

Diffuse sclerosis and Addison's Disease: Biochemical studies on gray matter, white matter, and myelin

Abstract Chemical studies were performed on the brain of a patient with a sex-linked form of diffuse sclerosis and Addisons Disease. As expected for a demyelinating disease, the major abnormality was seen in the white matter, and especially in myelin. The yield of myelin was significantly reduced, as were the contents of cerebroside, plasmalogen, and phosphatidyl ethanolamine. Cholesterol esters, while increased in white matter, were not detected in myelin. The fatty acid composition of the myelin glycolipids was abnormal, in that there was a considerable increase in the amount of short-chain (C 16 and C 18 ) fatty acids, and a significant reduction in the amount of long-chain and hydroxy fatty acids. It is likely that the long-chain and hydroxy fatty acids were located on the outer portion of the myelin sheath, which is damaged first in a demyelinating disease. Myelin regeneration probably occurs through biosynthesis of short-chain fatty acids, utilizing the soluble de novo system.

Vertigo in childhood. Comments on pathogenesis and management

The Bronx-Lebanon Hospital Center, Fulton Avenue at 169th Street, Bronx, N.Y. 10456. * From the Department of Pediatrics, The Bronx Lebanon Hospital Center. † From the Department of Otolaryngology, Albert Einstein College of Medicine. This work is supported in part by PHS Research Grant No. NS-10238-01 from the National Institute of Neurological Diseases and Blindness. V ESTIBI7LAR DYSFUNCTION in children can present itself in a variety of ways. In early infancy, delayed maturation or congenital dysfunction of the vestibular system may slow down motor development. From 16 months on, the most common cause of vestibular dysfunction is a self-limited disorder known as benign paroxysmal vertigo. From six years on, vestibular dysfunction is most often related to seizure disorders. Dizziness is a familiar and nonspecific symptom, whereas vertigo which is described by the patient as a sudden sensation of swirling of himself or of his surroundings is

Development of Head Control and Vestibular Responses in Infants Treated with Aminoglycosides

Forty‐three infants who had been treated with aminoglycosides for neonatal sepsis were tested for acquisition of head control and vestibular nystagmus induced by changes in position, perrotatory stimulation with a torsion swing, and caloric irrigation of the ear canals. 13 non‐treated premature infants served as controls: they provided normative data on acquisition of head control, postural control and vestibular responses. Nine of the 43 treated infants had multiple abnormalities on vestibular testing and delayed acquisition of head control, which suggests that vestibular dysfunction may contribute to delay in acquisition of head control. Infants with ‘static encephalopathy’ following neonatal sepsis treated with aminoglycosides should be tested for possible vestibular dysfunction.

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-Barre 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. Blood for DNA study revealed homozygous deletion mutation in exons 7 and 8 of the survival motor neuron gene. This diagnosis should be considered in every child under 1 year of age who presents with acute weakness because Guillain-Barre syndrome in this age group is rare.

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. Although treatment with botulism immunoglobulin is now suggested for the acute phase of infantile botulism, its use for recurrence is controversial.