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Biomedical subjects

J Maytal

Publications and source records attributed to J Maytal.

At least 37 records · Page 2Linked to original sources
PubMed1995

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. METHODS: A retrospective chart review was conducted of all children referred to the pediatric neurology clinic for evaluation of headaches over a 2-year period. Charts were reviewed for headache characteristics, clinical indications for performing CT and MRI studies, and imaging results. Particular attention was paid to evidence of brain tumors, vascular anomalies, or hydrocephalus. RESULTS: A total of 133 records were studied. Subjects ranged in age from 3 to 18 years. Most patients were diagnosed as having either vascular migrainous headaches (52%) or chronic tension headaches (21%). Other headache diagnoses were mixed tension-migraine, psychogenic, and post-traumatic. Headaches were unclassified in 25 patients (19%). Seventy-eight patients (59%) had brain imaging: 45 had MRI, 27 had CT, and 6 patients had both. In most cases, brain imaging studies were performed in patients with atypical headache pattern, presence of neurologic abnormalities during the headache, general symptoms (ie, weight loss or fatigue), or because of parents' or doctors' concerns about brain tumors. Cerebral abnormalities were found on brain imaging in four patients, but none indicated the presence of a treatable disease and all were deemed unrelated to the presenting complaint. Our findings of no relevant abnormalities in a series of 78 brain imaging studies indicate that the maximal rate at which such abnormalities might appear in this population is 3.8%. CONCLUSIONS: These results indicate that brain imaging studies have very limited value in evaluating headaches in pediatric patients without clinical evidence of an underlying structural lesion.

Adolescent↗
PubMed1994

Association of Wilms' tumor and intracranial hemorrhage due to arteriovenous malformation.

A high incidence of congenital malformations associated with Wilms' tumor has been recognized lately. Aniridia, hemihypertrophy, and urinary tract anomalies are among the most frequently reported. Anomalies of the central nervous system associated with Wilms' tumor are rare. We report a previously healthy 4-year-old girl who presented with an intracranial hemorrhage secondary to a ruptured arteriovenous malformation. A routine abdominal examination revealed a large right-sided Wilms' tumor. The question generated by the coexistence of these two uncommon disorders in one individual is whether there is a genetic or other relationship between them, or whether this is merely a coincidental occurrence. The question at this point is unanswered and must await demonstration of additional, similar cases.

Angiography, Digital Subtraction↗
PubMed1993

Neuroradiological manifestations of focal polymorphic delta activity in children.

To examine the neuroradiological and clinical correlations of focal continuous polymorphic delta activity (PDA) in children, we reviewed the records of patients with continuous PDA that was either focal or lateralized to one hemisphere. Of 2571 electroencephalograms (EEGs) performed between July 1, 1988, and December 31, 1990, a total of 125 records from 87 patients revealed continuous PDA. Eighty of these patients had neuroimaging performed either by computed tomographic scan (n = 59) or by magnetic resonance imaging (n = 38) within 8 weeks of the EEG. Twenty-two patients (28%) showed no abnormalities on neuroimaging, 16 patients (20%) showed diffuse abnormalities, and 42 patients (52%) showed focal abnormalities that correlated with the EEG findings. Most (18 of 22) patients with no neuroradiological abnormalities presented to the hospital with seizures from various causes. The presence of focal spikes or additional focal EEG abnormalities did not differ significantly among the three imaging groups. Generalized EEG abnormalities and multifocal spikes were significantly more common among the patients who had generalized abnormalities on neuroimaging. Focal continuous PDA without correlated changes on neuroimaging occurred in 48% of patients, which is much higher than what has been reported in adults.

Adolescent↗
PubMed1993

Progressive nemaline rod myopathy in a woman coinfected with HIV-1 and HTLV-2.

Nemaline-rod myopathy was recently reported in eight young males infected with human immune deficiency virus type 1 (HIV-1). A 41-year-old woman had a 2-year history of progressive proximal-muscle weakness. Muscle biopsy demonstrated the presence of nemaline rods, predominantly in type 1 fibers. She was coinfected with HIV-1 and HTLV-2, as evidenced by positive polymerase chain reaction and serology. There was no lymphopenia or CD4 lymphopenia, despite an abnormal T-cell subset ratio, high CD8 count, skin anergy, and depressed in vitro response to mitogens. This case raises the possibility that dual infection may play a role in the pathogenesis of the rare nemaline-rod myopathies of HIV-infected patients.

Adult↗
PubMed1992

Recurrent status epilepticus in children.

Status epilepticus is an uncommon but life-threatening seizure. Little is known about the risk of recurrent status epilepticus in patients who present with an initial episode. To determine the risk of recurrent status epilepticus in children, we prospectively followed 95 children, identified at the time of their first episode of status epilepticus, for a mean of 29.0 months (range, 4-60 months). The patients' ages ranged from 1 month to 18 years (mean, 4.6 years). The cause of the status epilepticus was classified as idiopathic (n = 24), remote symptomatic (n = 18), febrile (n = 29), acute symptomatic (n = 18), or progressive neurological disorder (n = 6). Sixteen children (17%) had at least 2 episodes of status epilepticus. The risk of recurrent status was 4% (n = 1) in the idiopathic group, 44% (n = 8) in the remote symptomatic group, 3% (n = 1) in the febrile group, 11% (n = 2) in the acute symptomatic group, and 67% (n = 4) in those with progressive neurological disease. Recurrent status epilepticus occurred primarily in neurologically abnormal children. While neurologically abnormal children accounted for 34% (n = 32) of all children with status epilepticus, they comprised 88% (n = 14) of the children with recurrent status epilepticus (p less than 0.001) and all 5 of the children with multiple (greater than or equal to 3) episodes of status (p less than 0.001). Fifteen of 16 children with recurrent status epilepticus were being treated with antiepileptic drugs at the time of recurrence. The morbidity and mortality of status epilepticus were low.(ABSTRACT TRUNCATED AT 250 WORDS)

Anticonvulsants↗
PubMed1992

Childhood onset cluster headaches.

Cluster headaches are rare in childhood. We identified 35 patients with cluster headaches starting at or before 18 years of age, including 7 patients with onset prior to age 10. All patients met the criteria of the International Headache Society for episodic or chronic cluster headaches. Patients experienced cluster headaches for as long as 20 years before seeking medical attention and required many medical contacts to establish the correct diagnosis. The clinical features of cluster headaches during childhood were similar to those which typically occur in adult life. Cluster headache patterns changed over 18 years of follow up. The frequency and duration of cluster periods increased in 14 subjects. The frequency of single headache attacks during cluster periods also increased in a similar number of subjects. We conclude that cluster headaches starting in childhood or adolescence closely resemble the adult form of the disease. In many patients, the frequency and duration of cluster periods and the frequency of the individual headache episodes increased over time. Cluster headache represent a treatable under-recognized cause of severe headaches in childhood and adolescence.

Adolescent↗
PubMed1991

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. In this sporadic case of female muscular dystrophy, the identification of dystrophin-deficient muscle fibers made it possible to establish an accurate diagnosis of DMD affected female.

Biopsy↗
PubMed1991

Lorazepam in the treatment of refractory neonatal seizures.

We report the results of treatment of intractable seizures with lorazepam in seven neonates. All of the patients were part of a prospective study, who failed to respond to 40 mg/k of phenobarbital. Lorazepam was given intravenously at 0.05 mg/k and repeated up to a total dose of 0.15 mg/k if necessary. The diagnosis of seizures and the efficacy of treatment was assessed clinically and by EEG during the administration of lorazepam in three patients and on clinical grounds in four patients. Six patients were full term and one was premature; there were five males and two females. Four patients had hypoxic-ischemic encephalopathy, two had intracranial hemorrhage, and one had bacterial meningitis. Two patients received one dose of lorazepam, three received two doses, and two received three doses. Six patients responded with a complete cessation of seizures within three minutes of their last dose; the remaining patient (who received two doses) had a reduction in seizures. No patients developed apnea or hypotension during or immediately after the infusion of lorazepam and no other adverse effects were observed. Four patients remained seizure-free for the rest of the neonatal period and no other anticonvulsant medications were added. Seizures recurred in one patient at 16 hours; subsequent intermittent seizures were managed with additional phenobarbital. In another patient, seizures recurred at 12 hours and subsequent intermittent seizures were managed with phenytoin. In one patient, seizures continued with reduction of frequency and duration. We conclude that lorazepam may be effective in the treatment of neonatal seizures refractory to phenobarbital and that further treatment with intravenous phenytoin may be unnecessary under these circumstances.

Drug Therapy, Combination↗
PubMed1990

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 7.5-kb deletion of mitochondrial DNA documented in muscle, kidney, skin fibroblasts, and leukocytes, providing evidence for a multisystem mitochondrial cytopathy.

Acidosis, Renal Tubular↗
PubMed1990

Febrile status epilepticus.

As part of a study of status epilepticus in children (Maytal J, Shinnar S, Moshe SL, Alvarez LA. Pediatrics. 1989; 83:323-331); 44 children with febrile convulsions lasting more than 30 minutes were followed for a mean of 28 months (range 4 to 72). Thirty children were followed prospectively. Children with prior afebrile seizures or evidence of acute central nervous system infection were excluded. Nine (20%) children had prior neurological deficits. The duration of the febrile seizure was 0.5 to 1 hour in 41 cases (85%), 1 to 2 hours in 5 (10%), and greater than 2 hours in 2 children (5%). No child died or developed new neurological deficits following the seizures. The risk of recurrent seizures was increased, but only in the group with prior neurological abnormality. Six (66%) of these children had subsequent febrile seizures compared with 12 (34%) of the normal children (P = .08). Three (33%) had recurrent febrile status epilepticus compared with only 1 (3%) normal child (P = .023). The 2 children in the prospective arm of the study with recurrent febrile status epilepticus were both neurologically abnormal (P = .035). All 3 of the children who subsequently had afebrile seizures (2 prospective) were neurologically abnormal (P = .006 overall, P = .035 for prospective only). It is concluded that the occurrence of febrile status epilepticus in a neurologically impaired child is a risk factor for subsequent febrile as well as afebrile seizures. The occurrence of febrile status epilepticus in an otherwise normal child does not significantly increase the risk for subsequent febrile (brief or prolonged) or afebrile seizures in the first few years following the episode.

Child↗
PubMed1990

Risk of seizure recurrence following a first unprovoked seizure in childhood: a prospective study.

In a prospective study, 283 children who presented with a first unprovoked seizure were followed for a mean of 30 months from the time of first seizure. Subsequent seizures were experienced by 101 children (36%). The cumulative risk of seizure recurrence for the entire study group was 26% at 12 months, 36% at 24 months, 40% at 36 months, and 42% at 48 months. The cumulative risk of recurrence in the 47 children with a remote symptomatic first seizure was 37%, 53%, and 60% at 12, 24, and 36 months, respectively, compared with a cumulative risk of 24%, 33%, and 36% at 12, 24, and 36 months, respectively, in the 236 children who had had an idiopathic first seizure (P less than .01). In children with an idiopathic first seizure, the electroencephalogram was the most important predictor of recurrence. The cumulative risk of recurrence in the 81 children with abnormal electroencephalograms was 41%, 54%, and 56% at 12, 24 and 36 months, respectively, but only 15%, 23%, and 26% at 12, 24, and 36 months, respectively, in the 138 children with normal electroencephalograms (P less than .001). A history of epilepsy in a first-degree relative was a significant risk factor only in idiopathic cases with abnormal electroencephalograms. In children with a remote symptomatic first seizure, either a history of prior febrile seizures or the occurrence of a partial seizure were significant predictors of recurrence. Age at first seizure and duration of seizure did not affect recurrence risk in either the idiopathic or remote symptomatic group. A total of 84% of the children were not treated with antiepileptic drugs or were treated for less than 2 weeks. Only 9% were treated for longer than 3 months. Treatment did not affect the risk of recurrence. The results suggest that, even without treatment, the majority of children with a first unprovoked seizure will not experiment a recurrence. Children with an idiopathic first seizure and a normal electroencephalogram have a particularly favorable prognosis.

Adolescent↗
PubMed1989

Spontaneous spinal epidural hematoma in a 7-year-old girl. Diagnostic value of magnetic resonance imaging.

The authors report a 7-year-old girl who developed neck pain and stiffness over a four-day period. There was no fever, trauma, systemic illness or headache. Physical examination demonstrated subtle neurologic deficits indicative of cervical cord compression. CAT scan and subsequent Magnetic Resonance Imaging (MRI) of the cervical spine demonstrated a spinal epidural hematoma, which was evacuated surgically. Post-operative angiography failed to demonstrate a vascular abnormality. The child recovered without neurologic deficit. MRI proved to be a sensitive tool in identifying the nature and extent of this lesion, and may be considered in lieu of myelography.

Child↗
PubMed1989

Low morbidity and mortality of status epilepticus in children.

In an ongoing study of status epilepticus, 193 children with status epilepticus of varying causes have been followed up for a mean period of 13.2 months. Of these, 97 patients were recruited prospectively. The patients' ages ranged from 1 month to 18 years (mean, 5.0 years). The cause of the status epilepticus was classified as idiopathic in 46 cases, remote symptomatic in 45, febrile in 46, acute symptomatic in 45, and progressive neurologic in 11. The mortality and incidence of sequelae following status epilepticus was low and primarily a function of etiology. Seven children died within 3 months of having the seizure. New neurologic deficits were found in 17 (9.1%) of the 186 survivors. All of the deaths and 15 of the 17 sequelae occurred in the 56 children with acute or progressive neurologic insults. Only two of the 137 children with other causes sustained any new deficits (P less than .001). Duration of the status epilepticus affected outcome only within the acute symptomatic group (P less than .05). Neurologic sequelae occurred in 29% of infants younger than 1 year of age, 11% of children 1 to 3 years of age, and 6% of children older than 3 years of age. However, this was a reflection of the greater incidence of acute neurologic disease in the younger age groups. Within each cause, age did not affect outcome. Of the 193 children, 61 (32%) had a history of prior unprovoked seizures. Of the 125 surviving children with no history of prior unprovoked seizures, 37 (30%) had subsequent unprovoked seizures.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

EEG and brain death determination in children.

In a retrospective study involving several medical centers we identified 52 patients under age 5 years who met the adult clinical criteria for brain death and had at least one EEG with electrocerebral silence. Of the 52 patients, 31 died spontaneously and 21 were disconnected from the respirator. Repeat EEGs were obtained in 28 patients, and in all electrocerebral silence persisted. The study suggests that clinical criteria similar to those used for adults in the determination of brain death can also be applied to children above age 3 months and that a single EEG with electrocerebral silence is sufficient to confirm brain death in this age group.

Brain↗
PubMed1987

The coexistence of myasthenia gravis and myotonic dystrophy in one family.

We are reporting the unique coexistence of two distinct neuromuscular diseases, myotonic dystrophy and the juvenile form of myasthenia gravis, occurring in one family. A 16-month-old previously healthy female presented with a two month history of bilateral varying drooping of both eyelids and bilateral external ophthalmoparesis. The acetylcholine receptor antibodies were elevated, and there was a dramatic response to edrophonium confirming the clinical impression of myasthenia gravis. Spontaneous remission of the ptosis was noted after six months with no specific treatment. Many other family members were examined; none of them had clinical or laboratory evidence of myasthenia gravis. The clinical examination of the mother and the maternal grandmother, neither of whom had any complaints, resulted in a definite diagnosis of myotonic dystrophy. The proband's father and a 3-year-old sister were examined and found to be normal. We studied the HLA antigens of all of the available family members; none were found to have the HLA antigens most commonly associated with myasthenia gravis. Secretor gene studies were not helpful in providing additional genetic identification. The question generated by the coexistence of these two uncommon disorders in one family is if there is a genetic or other relationship between them or if this was merely a coincidental occurrence. At this point in time the question remains unanswered and must await demonstration of additional similar circumstances.

Female↗
PubMed1987

External hydrocephalus: radiologic spectrum and differentiation from cerebral atrophy.

External hydrocephalus (EH) is a condition in which infants with rapidly enlarging heads are found to have a CT scan that shows widening of the subarachnoid space with mild or no ventricular dilation. In this study, 74 infants with EH associated with a variety of conditions were examined clinically and with CT scans to identify the radiologic features of the condition as well as to understand its clinical and radiologic evolution. Some of these CT scans were compared with those of patients with cerebral atrophy. The characteristic CT findings in EH were bifrontal widening of the subarachnoid space and widened interhemispheric fissure frontally with only mild enlargement of the rest of the subarachnoid space. Occasional mild to moderate ventriculomegaly was also present. In most cases the radiologic findings could be distinguished from those seen in cerebral atrophy, even without the clinical history of an enlarging head. The cases of cerebral atrophy showed prominent sulci throughout without disproportionate bifrontal widening of the subarachnoid space. Two major groups of infants are identified. The largest group (47) consisted of infants with primary megaloencephaly. Megaloencephaly was idiopathic in 40 infants, many of whom (19) met the criteria for benign familial macrocephaly. Others (7) had a clearly defined genetic syndrome. The radiologic abnormalities in these infants usually disappear by 2 years of age. The other major group (14) consisted of infants who sustained CNS or systemic insults such as subdural hematomas (8) or meningitis (3), which cause impaired CSF absorption. Thirteen premature infants with EH were also identified. It is concluded that EH is an age-related self-limited condition occurring in infants with open cranial sutures and that usually resolves without intervention by 2-3 years of age.

Atrophy↗
PubMed1986

Idiopathic external hydrocephalus: natural history and relationship to benign familial macrocephaly.

External hydrocephalus was identified in 63 infants. The 36 infants in whom external hydrocephalus was idiopathic constitute the study population. The group was homogeneous. Although not all were macrocephalic at birth, the head circumference exceeded the 95th percentile in all cases by 1 year of age, and subsequent head growth was parallel to the 95th percentile. The head computed tomographic scan showed a characteristic picture of a prominent interhemispheric fissure, a collection of subarachnoid fluid over the frontal convexities, and prominent basal cisternae. Mild ventriculomegaly was present in ten cases. These abnormalities resolved after 18 to 24 months of age. No infant required therapy for the condition. Development was normal in 32 infants, suspect in three, and clearly abnormal in only one of the 36 infants. However, of the 32 infants with normal development, 14 were found to be delayed in gross motor development and five in language development at a prior time. There was a family history of macrocephaly in 88% of cases. Several families had a mixture of members with external hydrocephalus and benign familial macrocephaly throughout several generations. These findings suggest that idiopathic external hydrocephalus is a relatively benign, self-limited condition that resolves without treatment and is closely related to benign familial macrocephaly.

Black People↗