Acute motor axonal polyneuropathy after a cisatracurium infusion and concomitant corticosteroid therapy.

A 40-yr-old male was admitted to the intensive care unit following blunt chest trauma. He had multiple rib fractures, bilateral pneumothoraces, and acute respiratory failure requiring mechanical ventilation. Sedation was achieved with midazolam and morphine, and later with propofol. The patient was paralysed with a continuous infusion of cisatracurium 1.42-5.75 micro g kg(-1) min(-1). Methylprednisolone 125 mg i.v. every 12 h was also started. After discontinuation of the cisatracurium infusion 7 days later, the patient manifested a flaccid quadriplegia with absence of deep-tendon reflexes. No sensory deficits were observed. Electromyography (EMG), repetitive nerve stimulation testing, and single fibre EMG (SFEMG) were performed at regular intervals after stopping cisatracurium. Clinical symptoms and electrophysiological examinations supported the diagnosis of acute motor axonal polyneuropathy related to concomitant administration of cisatracurium and corticosteroid therapy.

Local steroid treatment in idiopathic carpal tunnel syndrome: short- and long-term efficacy.

A clinical and electrophysiological study evaluated the usefulness of local steroid therapy for carpal tunnel syndrome (CTS). To evaluate the efficacy of local steroid therapy 32 patients (53 nerves) were randomly assigned to one of two groups: one (27 nerves) received 15 mg methylprednisolone acetate injected locally and the other (26 nerves) received the same amount of saline solution. The injections were repeated after a week. Clinical and electrophysiological findings were evaluated, double blind, at regular intervals. A clear-cut efficacy of steroid treatment was found. Only 8% of nerves were not benefitted while a marked early improvement was observed in most of the nerves. In order to appraise the long-term effect of local steroid treatment on CTS, 53 patients (91 nerves) were studied and followed up by means of clinical and electrophysiological examinations performed every 2 months for 2 years. The benefit of steroid treatment was transient. About 50% of the nerves became worse within 6 months and 90% within 18 months. Only a small percentage (8%) of the nerves remained improved at the 2-years follow-up. The clinical features were not useful in foretelling the duration of the improvement, which appeared to be related to the antidromic SAP latency.

Muscle rearrangement in patients with hemiparesis after stroke: an electrophysiological and morphological study.

Skeletal muscle changes were evaluated in patients suffering from hemiparesis after stroke. Concentric needle EMG and single fiber EMG of the paretic gastrocnemius medialis muscle were performed. Maximal amplitude of H, T and M responses in calf muscles of both the affected and the unaffected sides were determined by usual electrophysiological techniques. Muscle biopsy of the lateral gastrocnemius muscle of the affected side was performed to determine the distribution of fiber types and fiber sizes. Fibrillation activity and positive sharp waves occurred in paretic muscles in patients with more recent hemiparesis while the duration of motor unit potentials was prolonged in patients with long-lasting disease. The H/M ratio was increased on the paretic side. The percentage of type 1 fibers was augmented in most patients with normal mean diameter and low atrophy factor. The percentage of type 2 fibers was reduced with decreased mean diameters and with a high atrophy factor. Such changes may be related to inactivity or transsynaptic degeneration of type 2 motoneurons as a consequence of the interruption of the corticospinal tract. Increased percentage of type 1 fibers may be due to a collateral reinnervation process or a motor unit type transformation.

Catamenial pneumothorax. A case report.

Catamenial pneumothorax (CPT) is a rare complication of systemic endometriosis, as shown by this classic case. A nonsteroidal antiinflammatory prostaglandin synthetase inhibitor was used to treat the patient.

A suspected case of trimethoprim/sulfonamide-induced localized exfoliation.

A variety of skin reactions have been associated with the use of sulfonamides. Most of the reactions are uneventful and abate with drug discontinuation. Rare but severe skin reactions can occur and include the Stevens-Johnson syndrome and exfoliative dermatitis. Dermal reactions appear to be the most commonly reported adverse effect in children. Drug continuance following the development of a mild skin rash may be associated with the development of a more severe dermal reaction. This report summarizes a case involving a six-year-old white girl who developed exfoliative dermatitis confined to the feet following a course of oral trimethoprim/sulfamethoxazole (TMP/SMX) for otitis media. Exfoliation is an uncommon cutaneous manifestation following sulfonamide use. Although usually a generalized process, it is unusual to find it confined to the feet. Unfortunately there is no useful diagnostic method that could specifically elucidate the cause of a dermatologic eruption. Available evidence suggest that TMP/SMX was the probable cause of our patient's rash. Adjunctive antihistamines and corticosteroids are often useful when pruritus is present. Severe reactions require vigorous supportive treatment.

Peripheral neuropathy in amyotrophic chorea-acanthocytosis.

We investigated involvement of the peripheral nervous system in 6 patients with amyotrophic chorea-acanthocytosis. Electromyographic and neurographic findings, and pathological changes as demonstrated by examination of biopsy specimens of muscle and sural nerve indicate that most patients had an axonal sensorimotor polyneuropathy with more pronounced involvement of the distal portion of the nerves. Results obtained in one patient raised the question of an anterior horn cell disorder.

Autonomic nervous system dysfunction in chronic uraemics on haemodialysis.

A battery of cardiovascular reflex tests were performed in 35 patients with chronic renal failure on intermittent haemodialysis. An impairment of parasympathetic control was found isolated (14%) or combined with a damage of sympathetic control of cardiovascular system (26%). Moreover, maximum conduction velocity along sensory and motor fibres of posterior tibial nerve was measured in 21 patients. Autonomic dysfunction and somatic neuropathy did not appear strictly related.

Effect of ACTH4-10 on nerve fiber regeneration after sciatic nerve crush in rabbits: an electrophysiological and morphological study.

Crush of sciatic nerve in rabbits supported by morphologic and electrophysiologic data was used to evaluate the effects of ACTH4-10 on nerve fiber regeneration. Treated animals showed a statistically significant higher regeneration rate than did control rabbits. Fiber density and mean diameters of myelinated nerve fibers were measured in semithin nerve sections at 1 and 3 cm distal from the crushed point at three different time points. Nerve fiber density results were higher in ACTH4-10-than in vehicle-treated rabbits. This difference showed an unequivocal trend and attained a statistically significant level in the sections 3 cm distal from the crush. ACTH4-10 seemed therefore to have a beneficial effect on nerve fiber regeneration.

Comparative analysis of autonomic and somatic dysfunction in chronic uraemia.

The relationship between autonomic dysfunction and peripheral somatic neuropathy was investigated in uraemics. The battery of autonomic tests included R-R interval variation test, deep breathing, Valsalva manoeuvre, heart rate and blood pressure responses to standing, and sustained handgrip. Maximum conduction velocity along sensory and motor fibres of the posterior tibial nerve was measured. An impairment of parasympathetic reflexes was more frequent than a sympathetic damage, but with no relationship to the degree of electrophysiological disturbances. Cardiovascular autonomic dysfunction and somatic neuropathy in uraemia result to be two different entities in incidence and perhaps in pathogenesis.

Electrophysiological study of the snout reflex in normal subjects and in parkinsonian and pseudobulbar patients.

The snout reflex appeared to be electrophysiologically present in a great majority of normal subjects. Nevertheless, a facilitation of the R2 responses of the snout reflex was found in parkinsonian patients while in pseudobulbar patients an augmentation of the R1 took place. The snout reflex, therefore, seemed to behave in a similar way as the blink reflex. In order to explain the reported findings the authors took into account the different effects of the lesions of the lateral and ventral motor system upon the oligo- and multisynaptic reflexes.

Blink reflex in hemiplegia.

Data about the influence of hemispheric lesions on the blink reflex are conflicting. 21 hemiplegic patients and 11 control subjects were investigated. The duration, latency and electric area of electrically evoked blink reflex responses were evaluated by common electromyographic techniques. A depression of the ipsilateral and the consensual late response after stimulation of the paretic side was the most evident finding. However, also a certain increase of the early response and a depression of the late response of the paretic side independent of the side of stimulation emerged. Concerning the parameters taken into account, the evaluation of the latency period seems to be the most significant and reliable. Determination of the electric area provides additional useful data which, however, may easily lead to mistakes.

Effects of steroid hormones on muscle reinnervation after nerve crush in rabbit.

The ability of an association of three steroid hormones to influence the reinnervation process and the trophism of rabbit muscles denervated by crush of the sciatic nerve was investigated. The beginning of reinnervation was established with electromyographic recordings from the tibialis anterior muscle. The distance from the site of crushing to the point where the motor nerve enters the tibialis anterior muscle was then measured in each animal, and the nerve regeneration velocity (mm/day) was calculated: a slightly but significantly higher (P less than 0.001) mean value was found in treated animals compared with untreated ones. When soleus and extensor digitorum longus (EDL) muscles were histochemically examined 50 days after lesion, a larger mean diameter of type 2c fibers was found in treated than in untreated animals, pointing out a possible useful effect of the treatment. On the contrary, the size reduction of EDL type 2b fibers was more pronounced in treated rabbits, indicating a catabolic influence of the drugs on this fiber type.

Single fibre EMG in 6 cases of botulism.

In 6 cases of mild botulinum intoxication, conventional EMG and single fibre EMG (SFEMG) were performed on admission to our ward (about 15 days after ingestion of the toxin) and 4, 8 and 14 weeks after admission. In 4 cases, conventional EMG resulted in abnormal findings; and they normalized 4 weeks later. On the first examination, SFEMG revealed in all cases but one the occurrence of potential pairs with abnormal jitter (above 50 mus). The % of the potential pairs with abnormal jitter ranged in different cases from 17% to 44%. Some of the potential pairs with abnormal jitter showed blockings; the occurrence of blockings was not strictly related to jitter value. Mean jitter value and % of potential pairs with abnormal jitter became progressively reduced with increasing time after intoxication. Nevertheless, in 4 cases slightly abnormal findings were still present after 4 months. The data obtained in the basal condition are in agreement with those reported by others. SFEMG findings relate fairly well to conventional EMG data and clinical status. SFEMG has proved to be a very sensitive method for studying the neuromuscular transmission defect in botulism and in obtaining further information on the course of the syndrome.

Familial oculopharyngeal muscular dystrophy with distal spread.

An Italian male aged 50 years with oculopharyngeal muscular dystrophy is reported. Eleven of his relatives, over a period of three generations, had ptosis, dysphagia, nasal voice and difficulty in walking. The distribution of muscle weakness in the propositus and in one of his sisters was proximal in the upper, but distal in the lower limbs, confirming the existence of a relationship between oculopharyngeal dystrophy and distal myopathy. The first muscle biopsy appeared normal except for some round-cell collections, whereas the second one, 5 years later, showed marked dystrophic changes. Some patients with oculopharyngeal dystrophy may apparently pass through a secondary muscular inflammatory stage.