Migraine-associated vomiting and asystole in a child.

A variety of symptoms that accompany migraine in the child and adult are mediated by the autonomic nervous system. Significant effects on cardiac rhythm are uncommon, but can be life threatening. We describe a 3-year-old girl in whom migraine-associated vomiting precipitated cardiac asystole which was effectively treated with a cardiac pacemaker.

Familial hemiplegic migraine with crossed cerebellar diaschisis and unilateral meningeal enhancement.

A 6-year-old boy with a family history of hemiplegic migraine had a hemiplegic migraine lasting for 6 days complicated by prolonged fever, lethargy, and two brief focal seizures. An acute single photon emission computerized tomogram (SPECT) demonstrated decreased blood flow in the symptomatic cerebral hemisphere as well as crossed cerebellar diaschisis not previously documented in migraine. Another unique finding was the MRI with enhancement of the meninges and pial vessels over the symptomatic cerebral hemisphere.

Mobius syndrome: electrophysiologic studies in seven cases.

Mobius syndrome is characterized by congenital facial diplegia, frequent impairment of gaze, variable involvement of other cranial muscles, and various musculoskeletal anomalies. The site of dysfunction remains debatable. We performed detailed electrophysiologic studies in 5 children and 2 adults with Mobius syndrome to better delineate the pathophysiology of this disorder.

Effect of cocaine metabolites on behavior: possible neuroendocrine mechanisms.

The predominant cocaine metabolites were tested for central nervous system effects by intracerebroventricular (ICV) administration in rats. We found two types of responses: cocaine, norcocaine (NC), benzoylecgonine (BE), and benzoylnorecgonine (Nor BE) produced stimulatory effects, whereas ecgonine methyl ester (EME) and ecgonine (EC) resulted in no specific effect or sedation. A novel metabolite interaction was revealed when rats were pretreated with EME, which inhibited both analgesia and seizures by subsequently administered cocaine.

In vivo pharmacokinetics and in vitro production of cocaethylene in pregnant guinea pigs.

Simultaneous exposure to cocaine and ethanol results in the formation of cocaethylene, an active metabolite of cocaine. The concurrent abuse of both cocaine and ethanol is common during human pregnancy, but the kinetics of elimination and formation of this ethyl ester of cocaine have not been studied during pregnancy in any species. In the late gestation guinea pig (61 to 63 days), cocaethylene, at doses of 2 to 4 mg.

Cocaine and benzoylecgonine constrict cerebral arteries by different mechanisms.

This study was designed to determine possible mechanisms underlying the vasoconstrictor activity of cocaine and its principal metabolite, benzoylecgonine (BE) in cat isolated cerebral arteries. The arteries constricted significantly in response to single doses of cocaine, BE and norepinephrine (NE; (P < 0.05).

Infantile spasms in children with Down syndrome.

The authors retrospectively identified 17 children with Down syndrome who developed infantile spasms, and analysed their etiology, EEG findings, response to medication, development and prognosis. Compared with symptomatic infantile spasms in the general population, which have a poor prognosis, these patients had a relatively benign outcome with regard to seizure control: only three of 16 survivors currently have seizures and seven of the 16 currently receive anticonvulsants. Developmental outcome was poorest in those with a superimposed hypoxic insult and in those who regressed developmentally at the onset of the spasms and did not regain developmental milestones.

Normal high-resolution cerebral 99mTc-HMPAO SPECT scans in symptomatic neonates exposed to cocaine.

Vascular constriction is said to account for a variety of clinical effects of cocaine. High-resolution 99mTc-hexamethylpropylene amine oxime single photon emission computed tomographic (SPECT) scans, which measure cerebral blood flow, were used to determine whether neonatal brain perfusion deficits are present in newborns with confirmed cocaine exposure. Normal, age-appropriate SPECT scans were found in 21 babies.

Cocaine metabolites in the neonate: potential for toxicity.

Recent reports indicate that cocaine metabolites have biologic activity and could be toxic. To explore this possibility, two studies were initiated. The first study aimed to define the distribution of cocaine species by quantifying levels of cocaine and its metabolites norcocaine, benzoylecgonine, and benzoylnorecgonine in newborn cord blood and meconium.

Childhood stroke and lupus anticoagulant.

Despite extensive evaluation, the cause of many childhood ischemic strokes remains unexplained. Two children are reported with stroke and lupus anticoagulant. Both had family members with features of the antiphospholipid antibody syndrome.

Möbius syndrome: evidence for a vascular etiology.

We report five infants with restricted lateral gaze, facial diplegia, feeding difficulty, and/or respiratory disorders without significant pulmonary disease. Viral studies were negative in all patients. Two children had radiologic findings that included brain-stem hypoplasia and symmetric calcification in the dorsal tectum at the junction of the midbrain and pons.

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.

Quantitation of benzoylnorecgonine and other cocaine metabolites in meconium by high-performance liquid chromatography.

A method for simultaneous extraction of cocaine and metabolites benzoylnorecgonine, benzoylecgonine and norcocaine from meconium was developed. The procedure uses solid-phase extraction columns with both cation-exchange and hydrophobic properties after vortex-mixing meconium with methanol. Chromatography utilizes reversed-phase high-performance liquid chromatography with a C18 column and phosphate buffer-acetonitrile as mobile phase.

An unusual cause of cerebral venous thrombosis in a four-year-old child.

Background And Purpose: Inherited protein S deficiency has been associated with an increased risk of thromboembolic disease. It is possible that such a coagulopathy could predispose children to the development of strokes by permitting clot formation in response to stimuli that ordinarily would be insufficient to cause thrombus formation.

Case Description: We evaluated a previously well 4-year-old boy who developed cerebral venous thrombosis after suffering minor head trauma.

Seizure exacerbation related to beta-lactam antibiotics in a child with cerebral dysgenesis.

A three-year-old boy with right hemimegalencephaly, developmental delay and left hemiparesis had well-controlled left focal seizures. Occasionally he had generalized seizures if he was given beta-lactam antibiotics. An EEG with simultaneous penicillin injection was performed to examine this apparent association: a secondarily generalized seizure was documented within two minutes of intravenous administration of the penicillin.

Seizures induced by the cocaine metabolite benzoylecgonine in rats.

The half-life (t1/2) of cocaine is relatively short, but some of the consequences of its use, such as seizures and strokes, can occur hours after exposure. This led us to hypothesize that a metabolite of cocaine may be responsible for some of those delayed sequelae. We evaluated the potential of the major metabolite of cocaine, benzoylecgonine (BE), to cause seizures.

Menstrual cycle-related fluctuations of tics in Tourette syndrome.

In a survey of 47 women of all ages with Tourette syndrome, 26% had a premenstrual increase in tics (P less than .001) when various hormonal states were examined. An increase in tics at menarche predicted the premenstrual tic exacerbation (P less than .

Effects of benzoylecgonine on the behavior of suckling rats: a preliminary report.

A major metabolite of cocaine, benzoylecgonine, causes behavioral activation that progresses to seizures when given intraventricularly to 2-week-old rats. The seizures are characterized by running, hopping, and vocalizing and are mixed with shorter-duration tonic episodes. Pretreatment with haloperidol, at a dose aimed at blocking stereotyped behavior, did not suppress these behaviors.

Hemimegalencephaly: clinical, EEG, neuroimaging, and IMP-SPECT correlation.

Iofetamine-single photon emission computed tomography (IMP-SPECT) was performed on 2 girls (5 1/2 and 6 years of age) with histories of intractable seizures, developmental delay, and unilateral hemiparesis secondary to hemimegalencephaly. Electroencephalography (EEG) revealed frequent focal discharges in 1 patient, while a nearly continuous burst suppression pattern over the malformed hemisphere was recorded in the other. IMP-SPECT demonstrated a good correlation with neuroimaging studies.

I-123 Iofetamine SPECT scan in children with neurological disorders.

I-123 Iofetamine (IMP) single photon emission computed tomography (SPECT) imaging of the brain in 42 patients (ages 14 days to 23 years) was compared with other localizing studies in children with neurological diseases. All had an EEG and at least one imaging study of the brain (computed tomography (CT) or magnetic resonance imaging (MRI), or both). Seventy-eight percent of the patients had an EEG within 24-72 hours of the IMP-SPECT scan.