Mycoplasma pneumoniae: a cause of coma in the absence of meningoencephalitis.

Mycoplasma pneumoniae encephalitis is a recognized cause of reversible coma in children. As an etiology of infectious encephalitis, it yields a relatively poorer prognosis than most other causes of infectious encephalopathies. Encephalitis is generally diagnosed by a constellation of clinical symptoms and confirmed by a cerebrospinal fluid (CSF) examination revealing cell pleocytosis and elevated protein. That Mycoplasma pneumoniae encephalopathy can occur in the presence of a normal CSF examination is less well appreciated. The authors report two children who presented with coma and normal CSF findings in whom a diagnosis of acute Mycoplasma pneumoniae infection was made. The two children both had rapid and complete recovery over several days. These cases exemplify that coma can result from acute infection with Mycoplasma pneumoniae in the absence of an inflammatory CSF response and that a normal CSF may herald a more favorable prognosis.

Late lateralizing and localizing EEG features of scalp-recorded temporal lobe seizures.

The authors studied the lateralizing value for temporal epileptogenesis of focal or hemispheric EEG ictal features that first appear > or = 5 seconds after hemispheric or diffuse EEG changes or after an artifact-obscured onset, by correlation with side of lobectomy abolishing or producing > or = 90% improvement of complex partial seizures (CPS). One hundred forty-eight seizures in 39 patients rendered seizure-free (SF) by lobectomy lacked early localizing features of which 91 (61%) seizures in 37 (95%) patients had late lateralizing or localizing phenomena. Eighty-seven (96%) of 91 late localizing or lateralizing seizures occurred ipsilateral to lobectomy, involving 35 (95%) of 37 patients. Late contralateral and shifting phenomena occurred in a total of 4 seizures of 2 patients. Among 17 non-SF but > or = 90% improved patients, 30 (48%) of 63 seizures lacking early localizing features showed late localizing or lateralizing phenomena. Of these, twenty (67%) involving 12 (71%) patients occurred ipsilateral to lobectomy. Among the remaining 5 (29%) patients, a total of 5 seizures (17%) were contralateral while 5 (17%) exhibited shifting laterality. These data indicate that seizures with late (> or = 5 seconds) localizing or lateralizing features cannot be discounted when assessing laterality of temporal epileptogenesis but cannot be used as the sole electrographic criterion.

Pathologic findings of multiple sulfatase deficiency reflect the pattern of enzyme deficiencies.

Multiple sulfatase deficiency is a rare metabolic storage disorder that manifests in childhood. It is probably an autosomal-recessive inherited condition, the gene for which has not yet been identified. Clinical features include mental deficiency and a dysmorphic appearance reminiscent of a mucopolysaccharidosis. Unlike most storage disorders, there are multiple deficient enzymes; all are sulfatases, hence the name of the disorder. Biochemical testing reveals accumulation of glycosaminoglycans, sulfatides, and gangliosides in the brain and other tissues of affected patients. In previous accounts of postmortem examinations, white matter histologic and biochemical pathologic findings similar to metachromatic leukodystrophy have been reported. Ganglioside accumulation, secondary to interference with degradative enzyme activity by the accumulating glycosaminoglycans also has been demonstrated. The authors report a case of multiple sulfatase deficiency with only mild deficiencies of the arylsulfatases but with severe deficiencies of iduronate sulfatase and heparan sulfamidase. Pathologic changes were more in keeping with a mucopolysaccharidosis, with minimal white matter changes and deposition of metachromatic material. The authors postulate that the mild leukodystrophic changes but striking features similar to a mucopolysaccharidosis are reflections of the pattern of enzyme deficiency. The pathology of multiple sulfatase deficiency therefore represents an overlap between a leukodystrophy and a mucopolysaccharidosis, with the relative contribution of each pattern apparently depending on the pattern of enzyme deficiency encountered in each patient.

Iron deficiency: a cause of stroke in infants and children.

Iron deficiency is a common pediatric problem affecting 20%-25% of the world's infants. Most commonly causing anemia, iron deficiency is also implicated in such neurologic sequelae as irritability, lethargy, headaches, developmental delay, and infrequently papilledema, pseudotumor cerebri, and cranial nerve abnormalities. Rarely has iron deficiency been recognized as a significant cause of stroke in the adult or pediatric populations. We report a series of 6 children, 6 to 18 months of age, who presented with an ischemic stroke or venous thrombosis after a viral prodrome. All patients had iron deficiency as a consistent finding among the group, and other known etiologies of childhood stroke were excluded. These patients provide evidence of a strong association between iron deficiency and ischemic events in children between 6 and 18 months of age.

Ischemic stroke in children: Relationship to unsuspected trauma.

Thirteen cases of ischemic stroke in children were seen and followed up between 1981 and 1990. Brain hemorrhage and strokes due to meningitis, leukemia, or other malignancy were excluded. In four, no cause was found. Three were related to trauma that preceded the stroke by up to 24 h. Two were due to cardiac emboli, which occurred during cardiac catheterization for dilation of stenotic valves, and two occurred spontaneously in association with mitral valve prolapse. One stroke was related to an inapparent neurocutaneous syndrome, and one was related to a hyperlipidemia. Follow-up showed no recurrence. School performance and developmental assessment was normal in 11 of 13 (one had a learning disability and the other had hyperactivity). Mild motor deficits persisted in five, were moderate in two, and were severe in one. The overall prognosis was best in children under 5 years.

Neurological complications of balloon angioplasty in children.

We report on the occurrence of cerebrovascular accidents as a possible complication of balloon angioplasty in children. The first patient underwent balloon angioplasty for aortic stenosis and subsequently developed a right temporoparietal infarct in the vascular territory of the right middle cerebral artery. The second patient developed a right temporoparietal infarct also in the vascular territory of the right middle cerebral artery following balloon angioplasty of native coarctation of the aorta. This experience suggests that, in addition to the previously reported complications of balloon angioplasty, the occurrence of a potential cerebrovascular accident must be considered in the risk-benefit analysis of this procedure.

Cerebral monitoring of somatosensory evoked potentials during profoundly hypothermic circulatory arrest.

The factors that influence the functional integrity of the central nervous system during clinical procedures involving profoundly hypothermic circulatory arrest (PHCA) have not been objectively evaluated. Intraoperative monitoring of somatosensory evoked potentials (SEPs) was performed in nine infants undergoing PHCA during repair of congenital cardiac anomalies to investigate the short-term effects of this intervention on neurophysiologic function. Latency prolongation of the primary cortical (N18,P22) and cervical spinal cord (N13) responses, reflecting slowing of neural transmission with hypothermia, occurred as a power function of decreasing systemic temperature (p less than .01). The cortical evoked response disappeared during profound hypothermia (less than 18 degrees C), remaining absent throughout the period of circulatory arrest and for a variable period of time after reperfusion. Regression analysis indicated that the time required for the recovery of the cortical evoked response on reperfusion was a linear function of the time-temperature integral of the arrest period (p less than .001) and the pH at the onset of circulatory arrest (p less than .001). Neurologic complications occurred in three patients and included cortical blindness (n = 2) and a generalized seizure disorder (n = 1). Visual dysfunction was not reflected in the intraoperative SEP recordings, whereas prolonged delay of SEP recovery, indicative of global central nervous system injury, was observed in the patient who experienced seizures after the surgery. This preliminary experience with SEP monitoring during PHCA suggests a role for this modality in determining the short-term effects of this procedure on neurophysiologic function. The recovery characteristics of somatosensory neural transmission appear to be modulated by the duration of, and temperature and pH maintained during, the arrest period.

Electrophysiological studies in five cases of abetalipoproteinemia.

Auditory brainstem responses (ABRs), visual and somatosensory evoked responses (VEPs and SEPs) and nerve conduction studies were conducted in 5 patients with abetalipoproteinemia. The ABRs were normal in all cases. The VEPs were of normal amplitude but of increased latencies in two patients. The four eldest patients had delayed cortical SEPs but normal peripheral sensory nerve conduction studies. The peripheral motor conduction velocities were normal in all cases. The peripheral sensory studies showed normal velocity when a response was seen; however, the amplitude of the response was often reduced or it was absent. The electrophysiological studies reported here support a model of axonal loss of large myelinated fibres with secondary demyelination in abetalipoproteinemia.