Obstetrical brachial plexus palsy (OBPP) outcome with conservative management.

Resurgence of neurosurgical intervention of obstetrical brachial plexus palsy prompted our review of 186 patients evaluated between 1981 and 1993, correlating clinical examination, electrodiagnosis, and functional outcome with conservative management. Eighty-eight percent had upper brachial plexus palsies, and 63% were mild. Forty-two infants required no long-term follow-up because they rated 1 or 2 on initial physical examination. Comparing first and last follow-up clinical findings of the remaining 149 patients, there was high agreement (correlation r= 0.81; P < 0.001). Pearson correlation of initial physical exam with electrodiagnosis at three intervals was relatively stable (r= 0.87, 0.88, 0.69). One hundred eight (72%) of the patients remained in their original severity groups. Thirty-three of 41 patients with discrepant follow-up scores improved by at least one category. Eight patients deteriorated. The natural pathophysiology and recovery of OBPP is presented.

Persistent pulmonary hypertension in newborn infants with cogenital myotonic dystrophy.

We describe two infants with congenital myotonic dystrophy that was complicated by persistent pulmonary hypertension. Both infants died of respiratory insufficiency that was unresponsive to ventilatory and pharmacologic support. One of the two infants was supported with extracorporeal membrane oxygenation before the diagnosis of congenital myotonic dystrophy was made.

Heterotopic ossification in children with burns: two case reports.

Heterotopic ossification is the formation of ectopic bone in soft tissue, and has been reported as a rare complication in pediatric burn patients. At our hospital, two 86% body surface area burn patients developed heterotopic ossification in the shoulder, elbows, distal femur, proximal tibia, fibula, and ribs approximately four months after the burn injury. These two rare and unusual cases are presented documenting the clinical involvement, radiological studies, laboratory data, as well as treatment of their heterotopic ossification. Discussion will focus on the incidence, diagnosis, pathophysiology, and treatment of heterotopic ossification in burn patients and how this information relates to the specific diagnosis and management of the complication of heterotopic ossification in the burn child.

Normal values for the soleus H-reflex in newborn infants 31-45 weeks post conceptional age.

Normal values for the soleus H-reflex were established in 83 preterm and term infants 31 to 45 weeks postconceptional age. Infants at conceptional ages 31 to 34 weeks (n = 30) had a mean H-latency (msec) of 19.2 +/- 2.16; infants 35 to 39 weeks (n = 26), 16.67 +/- 1.48; and infants 40 to 45 weeks (n = 27), 15.94 +/- 1.45. Simple linear regression shows a significant relationship between conceptional age and H-latency (Pearson correlation = .68, R2 = 0.47, f = 71.31, p = .0001). Adjusting H-latency values for leg length, the regression of H-soleus latency/leg length (msec/cm) against conceptional age provides an even stronger relationship (r = 0.81, R2 = 0.66, f = 154.5, p = .0001). These results reflect the degree of myelination in infants of increasing conceptional ages. They can indicate the gestational age of the infants and afford comparative data in the event of peripheral nerve pathology.

Lyme disease presenting with bilateral facial nerve palsy.

Facial palsy bilateral, or recurrent, suggests a myriad of diagnostic possibilities. An 11-year-old boy is described whose diagnosis remained elusive for several months. Clinical evolution and subsequent laboratory studies confirmed that he had Lyme disease. Literature review suggests that this disorder is ubiquitous in its manifestations. The diagnosis should be remembered in unexplained neurologic disorders, particularly in cranial and peripheral neuropathies.

Rehabilitation management of children with spastic diplegic cerebral palsy.

Spastic diplegia has been recognized as the type of cerebral palsy most frequently associated with prematurity. Due to constantly improving neonatal care in developed countries, more and smaller premature infants are surviving, and the number of spastic diplegic children can be expected to increase. This paper reviews the incidence, pathophysiology, and associated handicaps of patients with this type of cerebral palsy. The role of the physiatrist and aspects of traditional management are discussed. Recent advances in treatment of spasticity and lower extremity bracing are stressed as they seem to be particularly suitable to spastic diplegic patients.

Congenital muscular torticollis: results of conservative management with long-term follow-up in 85 cases.

A retrospective review of 277 patients with congenital muscular torticollis seen between 1970 and 1982 was conducted. In 85 cases this was supplemented by questionnaires and recent photographs, permitting a two- to 13-year follow-up. The first visit for 81.6% of patients was before six months of age. All were enrolled in a specific physical therapy program at the time of the first visit, unless they presented with severe torticollis after 12 months of age. Torticollis was mild to moderately severe in 90.6% of cases. Sternomastoid fibrotic nodules were present in 38.6%, more frequently in the more severe cases. Hip dysplasia increased in direct relation to severity and occurred in 10.5% of cases. At 12 months the torticollis had been conservatively resolved in nearly 70% of patients regardless of severity and presence or absence of focal fibrosis. Tenotomies were indicated in only ten children, eight of whom had first been seen after 12 months of age. Long-term sequelae were mild and consisted of craniofacial asymmetry, intermittent head tilt, and mild scoliosis. Developmental asymmetry or high tone due to limited mobility in the cervical spine were noted in 25.3% of infants initially and tended to subside with appropriate therapy. However, 11.8% of patients with long-term follow-up showed persistent functional asymmetry of the involved body side despite mild or moderate severity, early diagnosis, and complete resolution of the torticollis. Long-term observations indicate that congenital torticollis rarely requires surgical treatment.

The Holt-Oram syndrome: a case presentation and review of management parameters.

Holt-Oram syndrome (HOS) is a dominant inherited disorder which causes skeletal defects of the arms and heart disease. This report describes a 2-year-old child with HOS whose skeletal anomalies affected his functional capabilities and developmental sequence. A comprehensive evaluation of the child, which included radiographic, cardiac, gross and fine motor assessment, electrodiagnosis and consideration of surgery, resulted in a carefully formulated treatment plan. Review of the literature emphasizes the importance of selectively balancing conservative management with surgery for a satisfactory cosmetic result and optimal function.

Severe multicore disease associated with reaction to anesthesia.

Multicore disease is a nonprogressive myopathy. To our knowledge, in all previous cases, the clinical course has been benign with no deaths attributed to it. We describe a patient who presented as a floppy baby and remained weaker throughout his life than any other patients previously described. Biopsy findings were characteristic of multicore disease. However, at age 21/2 years, our patient developed congestive heart failure that was easily controlled with digitalis and diuretics. Shortly after cardiac catheterization, the patient developed a high, unexplained fever and died 26 hours later despite aggressive attempts at resuscitation. Therefore, patients with multicores in skeletal muscle may have severe weakness and may also have a predisposition to complications subsequent to anesthesia.

Electrodiagnostic tests in the detection of malignant hyperthermia.

Malignant hyperthermia (MH) can be a catastrophic and sometimes fatal reaction in certain susceptible individuals who are exposed to skeletal muscle relaxants such as succinylcholine and/or certain inhalation anesthetics such as halothane. In an attempt to develop a safe, simple in vivo test to identify the at-risk patients, repetitive nerve stimulation (RNS) and electromyographic (EMG) studies were performed on 9 patients with MH, 15 patients suspected of having MH, and 9 control subjects. Serial recordings were made following injections of caffeine sodium benzoate, succinylcholine, and halothane at the test site. Measurements were done on the amplitude of the evoked compound motor action potentials (CMAP), on RNS, and on the number of negative peaks of motor unit potentials (MUP) on maximum EMG recruitment. The results showed the effects of the drugs on amplitudes of the CMAPs to be significant (P less than 0.001) in all three groups, but no significant differences were found among the groups (P = 0.93). The negative peaks of MUP on EMG recording showed a significant difference upon introducing succinylcholine (P less than 0.001), and significant interaction effects of the treatment by the patient group. Preliminary comparisons of in vivo tests with in vitro contracture tests in nine patients showed good correlation.

Spinal muscular atrophy: experience in diagnosis and rehabilitation management of 60 patients.

Sixty patients with spinal muscular atrophy (SMA) are presented. Although the life span with Type I SMA (Werdnig-Hoffmann disease) may be short, children with the disease can be made more comfortable with appropriate medical care and parental support. Despite electrodiagnostic findings which are not always pathognomonic and muscle biopsies which can be difficult to interpret, the clinical presentation of these infants is so typical and the natural course of the disease so predictable that management can be telescopically designed. findings and muscle biopsies are more definitive. In these more benign forms of SMA, anticipatory medical management focuses on possible In SMA-II (Childhood) and SMA-III (Kugelberg-Welander), electrodiagnostic findings and muscle biopsies are more definitive. In these more benign forms of SMA, anticipatory medical management focuses on possible dislocating hips, scoliosis, pulmonary compromise, and sudden increase in weakness associated with intercurrent illness or prolonged immobilization due to fractures or surgery. Further considerations include maintenance of strength and endurance, independence in self-care, psychosocial, educational, and vocational endeavors. These more chronically affected children are eminently habilitable.

Developmental outcome of infants with grade III intraventricular hemorrhage.

To determine the developmental outcome of premature infants weighing 1,750 gm or less at birth and who had grade III intraventricular hemorrhage (IVH), we followed up ten infants with IVH confirmed by computed tomography (CT) and ten CT-negative control infants until they were 12 months corrected age. The infants were evaluated at three-month intervals with neurologic examinations; hearing, speech, and language assessments; Bayley testing; and evoked response studies. Normal criteria were defined in each area. Eight of the ten grade III IVH survivors had identifiable defects, with a predominance of motor deficits, as assessed by two or more parameters. Only three of the ten patients without IVH had two or more suspicious or abnormal assessments. Infants with grade III IVH may have widespread damage. A multidisciplinary approach to evaluating these patients is mandatory to determine the full extent of various deficits. Similar studies of infants with all degrees of IVH may help to define its full impact on their long-term development.

Brachial plexus palsy in neonates and children.

In 135 children with obstetrical palsy, clinical and electrodiagnostic studies on inital and serial evaluation aided in designing a conservative treatment program. Prevention of contractures, maintenance of muscle strength and reinforcing the child's awareness of the affected limb were predicated on the limiting factors of permanent denervation and agnosia. Instability of the scapula, scapulohumeral adhesions and elbow flexion with loss of active supination defy conventional range of motion exercises. Forced passive supination of the elbow may actually compound the problem of radial head dislocation and ulnar bowing. Because of the young age of most of the patients, only 7 have had orthopedic intervention. Rotational osteotomy of the humerus and transfer of intact muscles seem to improve function. Prior to selection of muscles for transfer, electromyography is advised. Later surgical treatment of the shoulder and elbow seems to improve cosmesis but not function. Of the 135 children, 70% had mild residua, 22% showed moderate impairment and 8% had severe deficit.

Malignant hyperthermia and central core disease in a child with congenital dislocating hips.

We describe a development of a malignant hyperthermia (MH) syndrome, partially aborted by therapy, in a child with central core disease and congenital dislocating hips. Patients with central core disease appear to be more susceptible to MH; possibly those with elevated serum creatine phosphokinase levels, as in our patient, are especially susceptible. We review the clinical and pathologic aspects, possible pathogenesis, and treatment of the MH syndrome. An increased calcium level within the muscle fiber is suggested as the major cytodestructive factor, and that increase could be consequent to a plasmalemmal susceptibility to the provoking drugs hypothesized to be the basic defect in MH. Prevention of the full manifestations of MH is predicated on (1) a high index of suspicion in the search for history of anesthetic complications in the patient and his family, with or without evident neuromuscular disease, (2) recognition that there is a somewhat greater risk of MH developing in a patient who has certain "musculoskeletal" abnormalities or muscle weakness but that is not-except for central core disease-a classic clinicopathologically defined disease, (3) close monitoring of patients during anesthesia, and (4) if the syndrome develops, prompt therapeutic measures, including cessation of anesthesia.

Spontaneous potentials in premature and full-term infants.

Spontaneous potentials may be normally found in the electromyograms of premature and full-term infants. These potentials tend to disappear as the infants mature past three months of age. However, in some infants with cerebral or metabolic disorders these spontaneous potentials may persist. Their pathogenesis is obscure. The electromyographer must be aware of their existence in disorders unrelated to the lower motor neuron.