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%. In two males, the aetiology was traced to Melkersson-Rosenthal syndrome and these patients were separated from the rest of the group. Three children had two episodes of facial nerve paresis which completely resolved clinically within several weeks. Six other children underwent electrophysiological studies. Two of the latter with residual neurological damage, and one child with abnormal blink reflex only, showed decreased facial nerve conduction velocity and abnormal blink reflex. Three children with complete recovery had disturbed blink reflex only with normal nerve conduction. Brain imaging studies as well as laboratory work-up were non-contributory in all cases. CONCLUSION: The frequency of recurrent facial nerve palsy in children was similar to that in adults. The most significant factors in the evaluation of recurrent facial nerve palsy are medical history and physical findings at diagnosis and after short follow-up. In our patients, electrophysiological studies did not have either clinical or prognostic significance. The rate of full clinical recovery is about 70%, lower than in Bell palsy.

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. At follow-up, none of the patients had dynamic supination deformity during ambulation. All showed active contraction of the transferred tibialis anterior tendon. There was no case of overcorrection. Functional and cosmetic results were assessed by parents and surgeons as being excellent. Tibialis anterior tendon transfer is recommended to correct residual dynamic supination deformity and to restore muscle balance after satisfactory correction of idiopathic club foot contractures.

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. Eligibility criteria for inclusion in the study were: predominant sciatica, no motor or sphincteric involvement, unilateral disc herniation at the L5-S1 level on CT or MR imaging, age between 20 and 50 years. H-reflex responses were recorded bilaterally from the gastrosoleous muscle following stimulation of tibial sensory fibers in the popliteal fossa. H-reflex amplitude in millivolts (HR-A) and H-reflex latency in milliseconds (HR-L) were measured from the spinal reflex response. Pre- and post-manipulation measurements were compared between the affected side and the healthy side. Statistical evaluation was performed by the Wilcoxon matched-pairs test (SPSS). Thirteen patients displayed abnormal H-reflex parameters prior to lumbar manipulation, indicating an S1 nerve root lesion. The mean amplitude was found to be significantly lower on the side of disc herniation than on the normal, healthy side (P = 0.0037). Following manipulation, the abnormal HR-A increased significantly on the affected side while the normal HR-A on the healthy side remained unchanged (P = 0.0045). There was a significant difference between latencies on the affected side and those on the healthy side (P = 0.003). Following manipulation there was a trend toward decreased HR-L. However, this trend did not reach statistical significance (P = 0.3877). Eight patients displayed no H-reflex abnormalities before or after manipulation. Their respective HR-A and HR-L values did not change significantly following manipulation. Three additional patients were excluded due to technical difficulties in achieving manipulation or measuring spinal reflex. These observations may lend physiological support for the clinical effects of manipulative therapy in patients with degenerative disc disease.

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. As patients with CF are often deficient in vitamin E despite daily supplementation and normal serum levels, the authors suggest that the abnormal BAEPs demonstrated in the present study may reflect prolonged intracellular vitamin E deficiency. This finding suggests that BAEP studies may be useful in the neurological evaluation of patients with CF.

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.

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. Every child aged less than 8 years showed failure of posterior lumbar and/or sacral fusion. With growth, fusion increased so that at age 14 years 50% had a normal fusion and 50% had spina bifida occulta. At age greater than 16 years, the incidence was similar to that in the general population. In Perthes' disease, growth in fusion of the lumbosacral elements is delayed.

[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. However, the children improved spontaneously over the years. During childhood all 3 had manifest steatorrhea. Serum cholesterol was between 39 and 100 mg/dl, while triglycerides were normal to high. Reevaluation during the past year revealed areflexia, deficiency of vitamins A and E and of apoproteins A and B, and prolonged PT time in 2 of the children. Electron and light microscopy of small intestinal biopsies revealed vacuoles in the enterocytes. Electrophysiological tests revealed major disturbances in sensory conduction and brain-stem function. These cases differ from those described in the literature. Although in hypobetalipoproteinemia, 1 of the parents would be expected to be heterozygous and have low serum levels of APO B, in this family the parents had normal levels. Their children had low levels of serum APO A, while in patients with hypobetalipoproteinemia the levels are normal. There is a report of a case of deficiencies of both apolipoproteins, but the patient was asymptomatic, had chylomicronemia after a prolonged fast, and lower cholesterol levels than our patients. 8 other cases of apolipoprotein deficiency have been reported with biochemical characteristics similar to those of our patients, but with retention of chylomicrons in the small intestine.

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. The recovery period after cessation of N2O was characterized by a successive increase in the number of bursts and a simultaneous decrease in the silent periods of the EMG to a state similar to the baseline recorded prior to inhalation of N2O/O2. The H-reflex also was found to decrease gradually during inhalation of N2O/O2 to about one-half of the baseline. This parameter also returned to baseline after N2O wash-out. Our findings demonstrate quantitatively the effectiveness of N2O in reducing the central motor neuron pool excitability to an extent that suppresses involuntary movements and allows more controllable and comfortable dental management.

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. Contractures and bizarre deformities develop at an early stage and necessitate repeated orthopaedic intervention. The most likely etiology appears to be a vascular insult to the spinal cord.

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. The OORR and especially its late response were normal in a patient with acromegaly who did not experience sleep apnea. In two patients with sleep apnea, but without acromegaly, the late responses of the OORR were abnormal. It is suggested that the presence of abnormal OORR in sleep apnea may reflect a basic defect in pontomedullary control of respiration during sleep.

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. The pattern suggested autosomal recessive inheritance.

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. The disease consisted of external ophthalmoplegia, ptosis, and severe sensory and motor peripheral neuropathy. Three patients also had neuronal hearing loss. There was no evidence of central nervous system involvement and all patients were mentally intact. The combined disease was confirmed by radiologic, electrophysiologic, and histologic studies. Specific nutritional deficiencies, toxic elements, and systemic diseases affecting both the gastrointestinal tract and the nervous system were ruled out. It seems that these patients suffer from an autosomal recessive, presently unrecognized variant, of chronic neuropathic intestinal pseudoobstruction. In a patient with severe peripheral neuropathy of unknown etiology associated with symptoms suggestive of intestinal obstruction, the possibility of chronic neuropathic intestinal pseudoobstruction has to be considered.

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. After intramuscular injections of vitamin E the ataxia disappeared and electrophysiological variables reverted to normal.

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. This spectrum differs from that of the squid axon, in which depolarization produces only an increase in transmission over the whole range of wavelengths (Ross et al. 1977. J. Membr. Biol. 33:141-183). When wave a was measured at the edge of the fiber to obtain more signal from the surface membrane, the spectrum did not seem to differ markedly from that obtained from the entire width of the fiber. Thus, the difference in the spectrum between the squid axon and the vertebrate muscle cannot be attributed to the presence of the tubular system.