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Biomedical subjects

U Kramer

Publications and source records attributed to U Kramer.

71 records · Page 4Linked to original sources

Pseudoseizure manifestations in two preschool age children.

We report two patients with epilepsy with pseudoseizures at age 6 years. Both presented with intractable staring spells. Pseudoseizures were provoked and aborted by suggestion, leading to the diagnosis. In both patients, evidence of a neuropsychological disturbance was later found and psychotherapy started. Monitoring of intractable staring episodes is recommended prior to escalating antiepileptic drug levels or resorting to polytherapy. In addition, differentiation from other non-epileptic phenomena is necessary to initiate proper therapy.

Anticonvulsants↗

Use of subdural grids and strip electrodes to identify a seizure focus in children.

For patients with intractable seizures, the best surgical outcome is achieved following precise localization of the seizure focus. Scalp EEG monitoring may be insufficient and chronic subdural invasive EEG monitoring has therefore been advocated. At Children's Hospital in Boston, 31 children had chronic subdural monitoring from January 1990 through June 1994. The average age at implantation was 11 years. Most patients (22) had placement of grid electrodes combined with strip electrodes to map temporal and/or frontal regions bilaterally. Twenty of the patients eventually had a resective procedure based on the findings. During monitoring, cortical stimulations were performed to localize speech and somatosensory areas. There was only one complication, a subdural hematoma in a patient who had had previous surgery. Chronic subdural EEG monitoring is helpful in precisely localizing seizure foci in pediatric patients; it also allows motor and speech mapping and appears to be a safe modality in children.

Adolescent↗

Muscle imbalance in the aetiology of idiopathic club foot. An electromyographic study.

We performed electrophysiological studies on both legs of 52 children, aged from 3 months to 15 years, with idiopathic club foot. In only nine (17%) was no abnormality found. Isolated peroneal nerve damage was seen in 14 (27%). Abnormality of both peroneal and posterior tibial nerves was found in five (10%). Four patients (8%) had evidence of isolated spinal-cord dysfunction, whereas combined spinal-cord and peripheral-nerve lesions were seen in 14 (27%). Six patients (11%) had variable neurogenic electrophysiological patterns. In 13 patients in whom the studies were repeated neither progression nor improvement of the electrophysiological parameters was observed. Pathological electrophysiological findings were found in 66% of conservatively-treated patients. In the 43 patients treated surgically, all 16 with fair and poor results had pathological electrophysiological findings and 12 required further operations. Multiplicity of the pathological findings was related to the severity of the deformity of the foot; normal studies represent a good prognostic sign. Electrophysiological studies are useful in idiopathic club foot with residual deformities after conservative or operative treatment. Our findings support the theory that muscle imbalance is an aetiological factor in idiopathic club foot.

Adolescent↗

Technetium-99m HmPAO brain SPECT and outcome of hemispherectomy for intractable seizures.

With recent descriptions of the modified hemispherectomies and hemicorticectomy, there has been renewed interest in hemispherectomy for treatment of intractable seizures with hemiparesis. Because long-term outcome remains uncertain, patient selection remains difficult. 99mTc-HmPAO brain SPECT has been a helpful adjunct in the evaluation of epilepsy surgery candidates. We report SPECT scan findings in 7 patients who underwent hemispherectomy and compare these results with scalp EEG findings. Six patients had unilateral SPECT findings and all had a favorable outcome, regardless of surface EEG findings.

Adolescent↗

West syndrome following deep hypothermic infant cardiac surgery.

Postoperative seizures are among the more common complications of cardiac surgery in children. These seizures have traditionally been considered benign, transient phenomena with little, if any, prognostic significance. We report 4 infants with early postoperative seizures following cardiac surgery who later developed the previously unreported complication of West syndrome, with infantile spasms, hypsarrhythmia, and developmental delay. This group constitutes 6% of 67 infant spasms evaluated over a 5-year period at Boston Children's Hospital. The postoperative seizures in these 4 patients were more difficult than usual to control with antiepileptic therapy; otherwise no intra- or perioperative features distinguished these infants who later developed West syndrome from infants with apparently benign "postpump seizures."

Adrenocorticotropic Hormone↗

Drop attacks induced by hypothyroidism.

Drop attacks (DA) are defined as sudden repeated falls without warning and without loss of consciousness. A variety of etiologies are known to be responsible for symptomatic DA, but the reason for idiopathic DA remains obscure. A 70-year-old woman who suffered from classic DA over a period of a year is described. The diagnostic work-up revealed severe myxedema. Treatment with eltroxin resulted in complete clinical and endocrine recovery and disappearance of DA while with discontinuation of hormone replacement therapy, DA reappeared. Thus, a direct relationship between hypothyroidism and DA can be considered.

Accidental Falls↗

Synovial copper deposition as a possible explanation of arthropathy in Wilson's disease.

A patient with longstanding Wilson's disease and arthritis is presented. Synovial biopsy disclosed thickening of the membrane, intimal histiocytes, and lymphocyte infiltration without pigmentation. X-ray energy spectroscopy demonstrated copper and iron in high concentrations. These findings may contribute to our understanding of the development of the arthropathy in patients with Wilson's disease.

Arthritis↗

[Aspects of rehabilitation after early stabilization of complex injury of the cervical spine].

Within the scope of catamnestic investigation and a follow-up examination 170 fractures of the cervical spinal cord, operated in a period of 8 years, were explored. Dependent on the neurological starting point the length of stay in hospital, the time of temporary disablement and the results of social rehabilitation were evaluated. The examination results are appraised.

Activities of Daily Living↗

[Syringomyelic form of leprosy].

A 60-year-old man suffered from recurrent painless burns. During his first neurological work-up, ulcers with trophic changes were noted on the dorsum of both hands. Temperature and pain sensation were decreased over the upper extremities, but touch, vibration and position sense were normal. Syringomyelia was considered, but neuroradiologic evaluation was negative. A year later he was readmitted and was found to have patchy anesthesia involving all extremities, but no distal muscle weakness or wasting; tendon reflexes were normal. Leprosy was diagnosed and confirmed by skin and nerve biopsies. This syringomyelic form of leprosy is difficult to diagnose as it often mimics intraspinal space-occupying lesions. In Israel, where leprosy is still endemic, unusual sensory disturbances, such as patchy dysesthesia, should suggest its possibility.

Diagnosis, Differential↗

Hypoglycaemic effect of proinsulin and insulin in intact and eviscerated rats.

The hypoglycaemic potency of biosynthetic human proinsulin and human insulin was studied in intact anaesthetized rats and in eviscerated hepatectomized rats, the latter serving as model for an in vivo muscle/fat tissue preparation. Intravenous dose response curves demonstrated a similar about 5-times smaller potency of proinsulin in both experimental conditions (half-maximal effective dose in intact rats for insulin 3.5, for proinsulin 17.5 nM/kg body weight; in eviscerated rats for insulin 3.6, for proinsulin 18.5 nM/kg). In intact rats hepatic glycogen content 2 h after hormone injection was higher in proinsulin than in insulin treated animals despite similar preceding hypoglycaemia which might suggest a preferential inhibitory action of proinsulin on hepatic glycogen breakdown (135 mumol glycosyl units/g liver for proinsulin-injected and 82 mumol glycosyl units/g liver for insulin-injected rats at half-maximal hypoglycaemia).

Animals↗

[Perimembranous glomulonephritis in heapatitis associated with hepatitis B antigen (author's transl)].

Hepatitis B (HB) antigen was persistently found in two children with perimembranous glomerulonephritis. Soluble immune complexes were demonstrated in both instances. These circulating immune complexes were found only in serum fractions enriched with HB antigen. Immunohistology of sections of kidney demonstrated immune complexes in a granular pattern in the subepithelial regions of the glomerular capillary walls. Fluorescent HB antigen was found in the same region. HB antigen was also detectable in urine. Participation of the complement system in the progress of the disease was demonstrated quantitatively by a decrease in complement factors C1q, C4 and C3 in peripheral blood. Histological, including electromicroscopic, studies in both cases revealed diffuse thickening of the glomerular basement membrane with dense deposits of different extent, and spikes of the basment membrane. These studies strongly suggest an interrelationship between hepatitis with HB antigen and glomerulonephritis.

Adolescent↗

Clinical categorization of 312 children with chronic headache.

Three hundred and twelve children referred to an outpatient pediatric neurology clinic, with headache that lasted more than 3 months, were retrospectively reviewed. On average, the age of pain onset was 8.4 years. Migraine was diagnosed in 54% of these children and tension-type headache was found in 22% of those with chronic headache. Most children (85%) had common migraine, while classic and complicated migraine was found in only 8.8% and 5.3%, respectively. Brief headaches, lasting from seconds to a few minutes, were found in 5.1% of the children evaluated. In this subgroup, a high rate of epileptic EEG activity was found. Out of 110 children who had undergone computerized tomography, only one was pathological (posterior arachnoid cyst). Our results indicate that chronic and recurrent headache without accompanying neurological symptoms are usually benign and therefore in most cases neuroimaging is not indicated.

Adolescent↗

The value of EEG in children with chronic headaches.

To establish the usefulness of electroencephalography (EEG) as a diagnostic tool in the evaluation of headaches in children, we retrospectively reviewed the records of all children referred to our outpatient neuropediatric clinic because of recurrent headaches. Of 312 children, 257 (82%) underwent EEG tracings: 143 of the children who had had EEG recordings were diagnosed as migraineurs. In 31 (12%) of the children, the EEG revealed epileptic activity. The highest incidence of epileptic EEG activity was found amongst the children with very brief headaches. In 22 (8.6%) of the children, diffuse or focal slowing was detected. The group with migraine headache had a significantly higher incidence of slowing than the group with other types of headaches. There was no correlation between focal EEG abnormalities and brain radioimaging studies or clinical course. We conclude that despite the high incidence of epileptic abnormalities, the contribution of EEG to diagnosis and treatment in children with chronic headache is minimal, and should not be routinely prescribed in these children.

Adolescent↗

Jittery babies: a short-term follow-up.

Thirty-nine full-term babies, appropriate for gestational age, and otherwise healthy, were followed-up in our neurology clinic because of jitteriness, until complete resolution of symptoms and neurological findings. The babies were examined at 3 month intervals, and were classified according to the severity of their associated neurological findings, hypertonicity and increased tendon reflexes, into two groups: 'mild' (n = 24), and 'moderate-to-severe' (n = 15). The mean follow-up period was 13.5 months. In 81% of the study population, jitteriness and neurological findings disappeared before the age of 9 months. In only 11% did they persist beyond the age of 1 year. The mean time until disappearance of associated neurological findings was significantly shorter (5.5 months) in the mild group, compared to the moderate-to-severe group (9.5 months). Only one infant displayed motor delay and required physiotherapy.

Child, Preschool↗