Recurrent facial nerve palsy in paediatric patients.

Unlabelled: The aim of the study was to investigate the clinical presentation and prognosis of recurrent facial nerve palsy (RFNP) in children. The files of 182 patients referred to the Schneider Children's Medical Centre of Israel for neurological evaluation of isolated peripheral facial nerve palsy between October 1992 and December 1998 were reviewed. RFNP was found in 11 patients (9 females, 2 males), with an incidence of 6%.

Tibialis anterior tendon transfer for residual dynamic supination deformity in treated club feet.

Twenty-seven previously treated club feet in 25 patients were evaluated retrospectively following tibialis anterior tendon transfer to the dorsum of the foot to correct residual dynamic supination deformity. In 11 feet, the transfer was combined with additional soft tissue and or bony procedures to treat other accompanying deformities. Electrophysiologically demonstrated peroneal weakness causing muscle imbalance contributed to the etiology of this dynamic deformity.

Spinal manipulation results in immediate H-reflex changes in patients with unilateral disc herniation.

The aim of this clinical investigation was to determine whether the abnormal H-reflex complex present in patients with S1 nerve root compression due to lumbosacral disc herniation is improved by single-session lumbar manipulation. Twenty-four patients with unilateral disc herniation at the L5-S1 level underwent spinal H-reflex electro-physiological evaluation. This was carried out before and after single-session lumbar manipulation in the side-lying position.

Impaired brainstem auditory evoked potentials in patients with cystic fibrosis.

Brainstem auditory evoked potentials (BAEPs) were measured in 11 young patients with cystic fibrosis (CF). Though none had clinical evidence of neurological impairment, all had various abnormal components of brainstem auditory evoked potentials (BAEPs). Abnormal BAEPs may be attributed to nutritional deficiencies including deficiencies of vitamins E and B6.

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%).

Perthes' disease and posterior lumbosacral union.

Delayed bone development is common in Perthes' disease, and affected children are usually smaller than normal. Somatosensory evoked potentials (SEP) studied in 25 cases of Perthes' disease were normal. Fifty-four patients with active or healed Perthes' disease were examined.

Transient suppression of involuntary movements in cerebral palsy patients during dental treatment.

The efficacy of nitrous oxide (N2O) and oxygen (O2) inhalation in reducing involuntary movements in cerebral palsy (CP) dental patients was examined using electromyography (EMG) and H-reflex techniques. Quantification and analysis of the EMG data revealed a progressive elimination of these movements to a point resembling the state of a normal muscle at rest. There was a gradual decline in the number of bursts and an increase in the silent periods of the EMG while under N2O.

[Familial hypobetalipoproteinemia with steatorrhea and malabsorption].

In a family in which the father was the mother's uncle, 3 of the 7 children were affected by a syndrome of malabsorption with various clinical symptoms. Diarrhea appeared in 2 of the children at birth, and in the third child at six months. The diarrhea led to failure-to-thrive, muscular wasting and abdominal swelling.

Nontraumatic, acute neonatal paraplegia.

Nontraumatic, acute neonatal paraplegia with associated lower limb vascular phenomena is a relatively rare condition. Three such cases are reported. The paralysis is primarily motor with minimal neurologic recovery.

Abnormal orbicularis oculi reflex response in sleep apnea secondary to acromegaly. Evidence of pontomedullary dysfunction in sleep apnea syndrome.

Severe sleep apnea was present in a patient with upper airway obstruction due to acromegaly. The study of orbicularis oculi reflex responses (OORR) disclosed a marked prolongation of the late response prior to tracheostomy. Following the surgical relief of upper airway obstruction, sleep apnea disappeared, and the latency of the late response of the OORR was dramatically reduced but failed to normalize.

Familial progressive neuronal disease and chronic idiopathic intestinal pseudo-obstruction.

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is characterized by recurrent episodes of bowel obstruction without mechanical cause. In five members of two Jewish-Iranian families, CIIP was associated with progressive neuronal disease, starting before age 30, with ophthalmoplegia, sensorimotor peripheral neuropathy, and hearing loss. There was no evidence of CNS involvement.

Involvement of the peripheral nervous system in temporal arteritis-polymyalgia rheumatica. Report of 3 cases and review of the literature.

Involvement of the peripheral nervous system is very uncommon in the temporal arteritis-polymyalgia rheumatica syndrome. Three different presentations of the involvement can be recognized: mononeuropathy, polyneuropathy and brachial neuropathy--C-5 radiculopathy. We report 3 patients in whom peripheral nerve symptomatology dominated the clinical picture of the disease, and review 20 previously published cases.

Familial intestinal pseudoobstruction dominated by a progressive neurologic disease at a young age.

Chronic neuropathic intestinal pseudoobstruction is a rare entity, characterized by recurrent episodes of bowel obstruction without a mechanical obstructive cause. We report five members of two Jewish-Iranian families in whom chronic neuropathic intestinal pseudoobstruction was associated with an identical and unique progressive severe neuronal disease. It appeared within the first two decades of life.

Hypovitaminosis E induced neuropathy in exocrine pancreatic failure.

A 4 year old girl with congenital nerve deafness and pancreatic insufficiency had incapacitating ataxia. Electrophysiological studies of the median nerve and the brain stem evoked response were abnormal. Serum vitamin E concentration was low.

Radial propagation of muscle action potential along the tubular system examined by potential-sensitive dyes.

Isolated single (Xenopus) muscle fibers were stained with a non-permeant potential-probing dye, merocyanine rhodanine (WW375) or merocyanine oxazolone (NK2367). When the fiber was massively stimulated, an absorption change (wave a), which seemed to reflect the action potential, occurred. Simultaneous recording of optical changes and intracellular action potentials revealed that the time-course of wave a was slower than the action potential: the peak of wave a was attained at 1 ms, and the peak of action potential was reached at 0.

Action potentials of isolated single muscle fibers recorded by potential-sensitive dyes.

Light transmission changes upon massive stimulation of single muscle fibers of Xenopus were studied with the potential-sensitive nonpermeant dyes, merocyanine rhodanine (WW375) and merocyanine oxazolone (NK2367). Upon stimulation an absorption change (wave a) occurred, which probably represents the sum of action potentials in the transverse tubules and surface membrane. In WW375-stained fibers wave a is a decrease in transmission over the range of 630 to 730 nm (with NK2367, over the range of 590 to 700 nm) but becomes an increase outside this range, thus showing a triphasic spectral pattern.

Structural changes in single muscle fibers after stimulation at a low frequency.

Direct stimulation of single muscle fibers from Xenopus laevis at a frequency of 1 Hz results in a decline of the peak isometric twitch tension after about 200 twitches. Fibers were chemically fixed in glutaraldehyde after a varying number of twitches and at several fatigue levels, and the ultrastructural appearance was compared with that of resting fibers treated by identical fixation methods. No gross structural abnormalities were observed but subtle changes occurred.

Intracellular localization of markers within injected or cut frog muscle fibers.

Many experimental procedures require drastic alterations of muscle fibers, such as cutting the fiber or injecting molecular probes through microelectrodes. We report the ultrastructure of similarly altered muscle fibers and the intracellular distribution of injected horseradish peroxidase (HRP). Cut fibers appear structurally normal at distances greater than 500 microM from the cut end, however, the structure deteriorates nearer to the cut.