Robert P. Cruse

Clinical outcome after complete or partial cortical resection for intractable epilepsy

This is the first epilepsy surgery series to analyze the definition of “completeness” of resection, based solely on results of chronic scalp and subdural EEG recording. When patients had complete removal of all cortical areas with ictal and interictal epileptiform discharges, the clinical outcome was usually good. When areas with epileptiform discharges were left behind, good outcome was significantly less frequent. This correlation between complete resection and good outcome was independent of the presence or absence of CT-detected structural lesions or sharp waves on post-resection electrocorticography. These results support completeness of resection, defined by prolonged extraoperative EEG, as an important factor in seizure surgery.

Hypothermia and barbiturate coma for refractory status epilepticus

Three pediatric patients with generalized status epilepticus unresponsive to therapy with conventional anticonvulsants were successfully treated with moderate hypothermia (30± to 31±C) and barbiturate coma with thiopental. All 3 patients were treated with thiopental at doses producing burst suppression or an isoelectric tracing on the EEG and thiopental and barbiturate levels were followed sequentially in the plasma. Continuous thiopental infusion rates of 5 to 55 mg/kg ± h maintained burst suppression and correlated with plasma thiopental levels of 25 to 40 mg/dl. Total doses of thiopental used to obtain and maintain burst suppression ranged from 15 to 50 g over 48 to 120 h. In all 3 patients, control of the status epilepticus was obtained. Moderate hypothermia and thiopental barbiturate coma are indicated in patients with generalized tonic-clonic status epilepticus which cannot be controlled with standard anticonvulsant drug therapy. This regimen has the advantage that the patient can be managed in an ICU without the need for general anesthesia with volatile anesthetic agents.

Gilles de la Tourette's syndrome Effects of stimulant drugs

We reviewed the medication histories in 200 children with Gilles de la Tourettes syndrome to investigate how frequently CNS stimulants may unmask a latent syndrome or worsen existing tics. Forty-eight patients had received stimulant drugs. Nine were treated before the onset of tics, but only four were still receiving stimulants when tics began. In 39 patients with preexisting tics, stimulants increased tics in 11, caused no change in 26, and decreased tics in 2. Behavior improved in 22 patients. Thirteen of these 22 had no increase in tics when stimulants were used. Cautious trials of stimulant therapy may benefit some patients with this syndrome.

The Mechanism of Nitrazepam-Induced Drooling and Aspiration

Nitrazepam, a benzodiazepine anticonvulsant, is used in the treatment of myoclonic, akinetic, absence, generalized tonic–clonic, and complex partial epilepsies.1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 A...

Vasovagal syncope: diagnostic role of head-up tilt test in patients with positive ocular compression test.

We investigated the relative merits of the ocular compression test and the head‐up tilt test to aid differentiation of syncope and seizures in young patients. Sixteen patients (10 males and 6 females) with a mean age of 14 ± 4.7 (SD) years (range 7–22 years) underwent graded head‐up till (15°, 30°, and 45° for 2 minutes each, then 60° for 20 minutes) following positive ocular compression testing defined as precipitation of asystole for at least 3 seconds (mean 5 seconds ± 2 seconds, range 3–12 seconds). Each patient presented with recurrent unexplained loss of consciousness (mean number of episodes 30 ± 45, mean duration of illness 52 ± 40 months), and seven patients were receiving anticonvulsant medications, three of these had normal EEGs. Eleven patients (69%) developed vasovagal syncope during head‐up tilt, reproducing their clinical episodes (systolic blood pressure decreased from 105 ± 10 mmHg to 84 ± 13 mmHg, diastolic blood pressure from 75 ± 9 to 22 ± 25 mmHg, and heart rate from 89 ± 13 beats/mm to 37 ± 20 beats/min). Asystole occurred in two patients during vasovagal syncope lasting 11 seconds in one and 16 seconds in the other, and, it was associated with myoclonic movements in both (convulsive syncope). Based on these findings, and given the perceived potential hazards of the ocular compression test, the head‐up tilt test may be a safer procedure that adds useful information to the diagnostic evaluation of these patients.

Headache and Subacute Sinusiris in Children and Adolescents

SYNOPSIS

Hypoglycemia in a child with congenital muscular dystrophy.

Since skeletal muscle is an important source of precursor for gluconeogenesis, it would not be surprising if carbohydrate metabolism was altered in some muscle disorders. We report a 7-10/12-year-old white male with congenital muscular dystrophy whose recurrent episodes of vomiting and dehydration were due to fasting hypoglycemia. He was found to have a blood glucose of 21 mg/dl with an episode of vomiting and dehydration. Diagnostic fasting replicated the symptoms and hypoglycemia. Associated laboratory findings included hypoalaninemia, ketonemia, and acidosis. With use of frequent feedings, there were no further episodes over 1-9/12 years of observation. To our knowledge this is the first report of hypoglycemia complicating muscular dystrophy. However, hypoglycemia may not be diagnosed because of the nonspecific nature of the symptoms. Hypoglycemia should be considered when children with reduced muscle mass develop otherwise unexplained vomiting.

GROWTH PATERNS AND ENDOCRINE STATUS IN CHILDREN WITH OPTIC NERVE HYPOPLASIA

Optic nerve hypoplasia (ONH) is known to be associated with hypothalamic-pituitary dysfunction including growth hormone deficiency (hGH-D), panhypopituitarism and diabetes insipidus (DI). Previous series have focused on specific subgroups and probably did not include the full range of patients with ONH.We have examined growth and endocrine data on 66 subjects (38M, 23F) with ONH aged 1 to 33 yrs (mean 9.5 ± 7.3 yrs). Several distinct patterns of growth and erdocrine faction were found: 1) Normal height arbitrarily defined as rank ≥ 5% ile was present in 68%. All 7 adults were in this group demonstrating that at least some with ONH achieve normal adult height. 2) Growth failure occurred in 21 subjects (32%). Its onset was 2 years in 10/21, between 2 and 3 years in 4/21, and unknown in 7/21. This age of onset is younger than reported by G. Costin, et al. Growth hormone therapy was able to normalize the growth in 2 subjects. Among this subgroup 6/21 were hypothyroid, 8/21 had definite cortisol deficiency and 3/21 had spontaneous hypoglyoemia. hGH-D was documented in 21% of all ONH subjects, significant DI was present in 11% of subjects. Two additional abnormal growth patterns ware noted. 3) Early or precocious puberty occured in 4/21 growth deficient children, Directing the observation of C.A. Huseman, et al. 4) Progressive obesity and normal growth with hGH-D similar to craniopharyngioma occurred in 2 subjects.Conclusions: 1) Children with ONH should be followed carefully for emergence of growth and endocrine abnormalities from infancy. 2) A brief period of normal growth associated with puberty or obesity should not mislead the pysician into assuming later growth will be normal.

1121 VALPROIC ACID IN THE TREATMENT OF INTRACTABLE SEIZURES

45 patients with intractable seizures are being treated with a new anticonvulsant, valproic acid (VPA). Two separate protocols are being followed. VPA was initially added to previous drugs.The first protocol involves 19 patients with simple or complex absence seizures. Maximum VPA dose is 60 mgm/Kgm/day. 6 hr EEGs were obtained initially, after two weeks of placebo and after 10 weeks of VPA. EEG analysis included the number and duration of electrical seizures. All patients show clinical improvement. 14 have a 75-100% reduction in clinical seizures. 5 are seizure free; none on less than 60 mgm/Kgm/day. In 6, clinical improvement cannot be correlated with the degree of EEG improvement.The second protocol involves 26 patients with mixed seizure types. Maximum VPA dose is 30 mgm/Kgm/day. Routine EEGs were done initially and after reaching stable maximum dosage, 13 patients are improved. 7 have a 75-100% reduction in clinical seizure frequency and 6 a 33-74% reduction. 3 patients are not improved. EEGs show no significant change. No patient is seizure free at a 30 mgm/Kgm/day dosage.VPA was effective against a variety of intractable seizure types. There was no consistent correlation between clinical and EEG improvement. No serious side effects or exacerbations of seizures occurred.

COMPUTERIZED TOMOGRAPHIC FINDINGS IN PATIFNTS WITH CHILDHOOD HEMIPLEGIA

In 1937, Dyke, Davidoff and Masson described nine patients with infantile hemiplegia who showed cerebral hemiatrophy, enlargement of the ventricle on the affected side, displacement of these structures toward the affected side, ipsilateral thickening of the skull and ipsilateral dilatation of the sinuses. The present study documents CT findings in patients with infantile hemiplegia; the CT correlate of the “DDM skull”.54 hemiplegic patients were studied, 34 congenital and 20 acquired. The frequency of complicated pregnancies, low hirth weights, mental retardation, seizure disorder, abnormal skull x-ray, and EEG abnormalities were similar to previous studies of hemiplegic patients.The CT Scans showed either the combination of hemispheric hypoplasia with ventricular enlargement and ipsilateral shift, or a pattern consistent with infarct. In the congenital group these patterns were equally present. In the acquired group infarct was present to a greater degree. No specific etiology could be ascribed to either pattern, although 5 of 6 patients with postnatal trauma showed infarct.It is concluded that CT Scanning is a useful procedure in documenting cerebral abnormalities in patients with either congenital or acquired hemiplegias. Further correlative studies with regards to etiology are needed.