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Biomedical subjects

A M Bye

Publications and source records attributed to A M Bye.

31 records · Page 2Linked to original sources
PubMed1990

Hereditary sensory neuropathy type II without trophic changes.

Three cases with sensory peripheral neuropathies are reported. Case 1 presented with scoliosis, and cases 2 and 3 presented with abnormal gait. None had trophic limb changes, evidence of weakness, or a tendency to self-mutilation and each had normal motor studies on neurophysiological testing. Sural nerve biopsies showed a severe loss of myelinated fibres and case 3 had evidence of denervation on muscle biopsy. These cases are presented as examples of hereditary sensory neuropathy type II. They are unusual in that they do not have trophic changes.

Adolescent↗
PubMed1989

Epilepsy surgery in Sturge-Weber syndrome.

A patient with Sturge-Weber syndrome developed seizures at the age of 4 years. At 13 years of age, she had intractable complex partial seizures with marked visual symptomatology. Interictal encephalograms showed bilateral slow activity, more marked over the right hemisphere with epileptogenic activity maximal in the right temporal region. Serial computerized axial tomography scans demonstrated evolution of bilateral occipital lesions with calcification and adjacent low density areas that were more marked on the right. Magnetic resonance imaging, angiography and neuropsychological evaluations were performed. An extensive resection of the right occipital lobe was achieved. One year after surgery, the patient has had seven brief seizures. Delineation of an epileptogenic focus and surgical removal of the lesion in patients with intractable seizures can now be considered in selected patients with bilateral central nervous system pathology.

Adolescent↗
PubMed1989

Selective epilepsy surgery in tuberous sclerosis.

A patient with tuberous sclerosis diagnosed at the age of 5 years developed an intractable seizure disorder characterized by complex partial seizures numbering 10-20/day. Interictal electroencephalograms (EEG) showed a right frontal epileptogenic focus. A computerized tomography scan demonstrated calcification in the right frontal region at two sites, periventricular calcification and multiple low density lesions. Neuropsychological assessment showed a verbal intelligence quotient (IQ) of 69 and a performance IQ of 88. Telemetry and video monitoring recorded 10 seizures during which the EEG showed flattening of ongoing sharp wave activity in the right frontal region. Electrocorticography further identified and localized epileptogenic tissue in the right frontal cortex and surgical removal of involved tissue and the adjacent two tubers was carried out. Twelve months after surgery the patient has had only two brief seizures.

Child, Preschool↗
PubMed1988

Cramps following exercise.

A 13-year old boy presented with a 10-year history of severe muscle cramps experienced an hour after prolonged exercise. There was no history of exercise intolerance or myoglobinuria. A muscle biopsy showed a lipid myopathy and a deficiency of muscle carnitine palmityl transferase. He has responded to a high carbohydrate, low fat diet with added carbohydrate intake preceding extensive exercise. Diagnosis of this entity before an episode of rhabdomyolysis is unusual.

Acyltransferases↗
PubMed1985

Multiple sclerosis in childhood: a new look.

Five patients (four female, one male) with multiple sclerosis were managed at the Hospital for Sick Children, London, between January 1978 and June 1983. The age at which first symptoms occurred varied from three to 14 years. Focal seizures occurred in three cases and an encephalopathic process in three cases, and there was evidence of progressive intellectual deterioration in all five children. Computed tomography showed low-density lesions in the white matter of all five. Electroencephalograms were abnormal in all cases, and in two the disturbance was severe. Oligoclonal bands were present in the cerebrospinal fluid of two children. Four of the five children had abnormal responses to pattern-reversal stimulus.

Adolescent↗
PubMed1985

Transient zinc deficiency in a full-term breast-fed infant of normal birth weight.

A full-term male infant was seen at age 5 months with symptomatic zinc deficiency. He was breast fed and the mother's milk zinc levels were low. The infant responded to oral zinc supplements and has continued to be asymptomatic for 12 months after their withdrawal. This is the first report of transient zinc deficiency in an otherwise healthy, breast-fed, full-term infant of normal birth weight.

Breast Feeding↗
PubMed1985

Symptomatic vitamin E deficiency in cystic fibrosis.

A girl with cystic fibrosis who developed a neurological syndrome probably secondary to vitamin E deficiency at the age of 10 years is described. The severity of the deficiency and the early development of neurological features probably result from reduced intraluminal bile salt concentrations in addition to the pancreatic insufficiency.

Child↗
PubMed1985

Prolactin and seizure activity.

Prolactin secretion after tonic-clonic seizures (10 patients), complex partial seizures (five) and non-epileptic attacks (three) was studied in a group of children aged between 0.3 and 14 years. Seven patients with other subcategories of seizure disorders were also studied. Eight children with tonic-clonic seizures exhibited post ictal concentrations of prolactin greater than 500 mU/l. One of the children, who responded on one occasion, did not do so on another. Three children with complex partial seizures had post ictal prolactin concentrations greater than 500 mU/l, while in two the increased values were more modest (390 mU/l and 420 mU/l). The timing of the peak post ictal prolactin concentration varied from less than 20 minutes to a prolonged plateau for three hours. Other seizure types--simple partial with motor signs (2), absence seizure (1), myoclonic seizure (1), minor epileptic status (3) (with one exception), and non-epileptic attacks (3) were not associated with post ictal concentrations greater than 500 mU/l.

Adolescent↗
PubMed1983

The obesity hypoventilation syndrome and the Prader-Willi syndrome.

Fourteen children with the Prader-Willi syndrome have been managed at the Royal Alexandra Hospital for Children between the years 1964-1980--twelve male, two female. Six male children developed features of the obesity hypoventilation syndrome. The age of onset of this complication ranged from 4.0 to 12.6 years. With one exception those children with the obesity hypoventilation syndrome were more obese than those without it. At the time of onset of the syndrome, five of six patients had weights greater than or equal to 6.5 standard deviations above ideal body weight. Those children without the obesity hypoventilation syndrome had a range of standard deviations 1.0 to 4.2 above the ideal body weight. In four of six cases weight reduction and a cardiac failure regimen resulted in reversal of the obesity hypoventilation syndrome. With two of the six children there had been cardiomegaly and increased pulmonary venous vascularity on x-ray at a chronological age of three months. Two of the six children died.

Body Weight↗
PubMedDate not supplied

Corpus callosotomy for intractable epilepsy: seizure outcome and prognostic factors.

We reviewed the outcome of corpus callosal section in 64 adult and pediatric patients to identify factors associated with a good outcome: 48% of patients had a favorable outcome for overall seizure frequency. Improvement was noted in several seizure types and was most likely for drop attacks, particularly in the setting of a unilateral focal cerebral lesion or a true generalized epilepsy of Lennox-Gastaut type. Poor outcomes for drop attacks were more likely if there was associated severe intellectual handicap or bilateral independent spikes on interictal EEG. Complex partial seizures (CPS), most commonly of frontal lobe origin, also responded favorably. The complications of callosal section were usually mild and transient. New focal seizures occurred in only 2 patients and were not as frequent or disabling as preoperative seizures types. A worthwhile improvement in seizure outcome was achieved by completion of the callosotomy in 6 of 10 patients with unsatisfactory results from anterior callosotomy.

Adolescent↗
PubMedDate not supplied

Complex partial seizures in young children.

We retrospectively analyzed the clinical manifestations of complex partial seizures (CPS) in children aged < or = 10 years using video and EEG telemetry and evaluated their course, investigations, management, and seizure status at follow-up. Seventeen patients with CPS were studied at the Prince of Wales Children's Hospital (POWCH) and Prince Henry Hospital (PHH) between 1987 and 1992. Because 15 of the 17 patients had intractable seizures, the population was selective. Mean age was 6.5 years; 4 patients were aged < or = 2 years. Clinical features were normal or mild intellectual handicap (13); hemiplegia (5); and infantile spasms preceding CPS (4); of these, 2 also had simple partial motor seizures. Structural abnormalities were noted on scanning in 9 patients. Eighty-seven seizures were reviewed. Mean duration of each clinical seizure was 59.7 s (total population), 108 s (subgroup 1, aged < or = 2 years), and 48.5 s (subgroup 2, aged > 2 years). Major ictal manifestations were auras (9), staring (9), autonomic changes (6), and automatisms (17). In subgroup 1, automatisms were simple and mainly oroalimentary and gestural. Two patients had no change in surface ictal recordings, and 2 had normal interictal EEGs. At follow-up, 8 patients were seizure-free for 6 months, 1 was partially controlled (more than two seizures a month), and 8 had intractable seizures (two or more seizures a month). Seven patients underwent operation for intractable epilepsy, and 4 achieved a class 1A outcome (Engel classification).

Automatism↗