Neurosarcoidosis in acute inpatient rehabilitation: a case study.

INTRODUCTION: Sarcoidosis affects the nervous system in ~5-10% of cases. Common presentations for neurosarcoidosis can include facial nerve neuropathy, optic neuritis, meningitis, seizure muscle weakness, and paresthesia. Due to the complex treatment of neurosarcoidosis, few reports exist involving patients' recovery in an acute rehabilitation setting. CASE PRESENTATION: We describe a case of neurosarcoidosis affecting the cervical and thoracic spinal cord in an individual with known Chiari I malformation and associated syrinx decompression. DISCUSSION: We discuss the diagnosis and treatment of neurosarcoidosis and clinical implications of acute rehabilitation on functional recovery.

Is there a relationship between somatic and autonomic neuropathies in chronic alcoholics?

We investigated the relationship between somatic and autonomic neuropathy in 40 chronic alcoholics. Electromyographic and neurographic studies of upper and lower limbs and a battery of six cardiovascular reflex tests were carried out. A score for somatic or autonomic neuropathy was calculated. All parameters were investigated for possible relationship with total life dose (TLD) of alcohol intake. Somatic neuropathy was detected in 25 patients (62.5%) and autonomic neuropathy in 13 patients (32.5%). Nineteen patients (47.5%) presented only a somatic neuropathy, six patients (15%) had only an autonomic neuropathy, and seven (17.5%) had a combined somatic and autonomic neuropathy. TLD was significantly higher in the group of patients with combined neuropathy than in the group with isolated somatic neuropathy. There was no significant correlation between laboratory parameters of somatic and autonomic neuropathy. Our findings do not support the existence of a parallel involvement of peripheral somatic and autonomic cardiovascular nerve fibers in chronic alcoholism.

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.

Limb-girdle myasthenia: clinical, electrophysiological and morphological features in familial and autoimmune cases.

Limb-girdle myasthenia is an uncommon disease and includes familial and autoimmune forms. Patients present proximal muscle weakness and wasting, and sometimes fatigability, without cranial nerve involvement and fluctuations. We observed, during a 15-year period, nine subjects with limb-girdle myasthenia, (24-55 years; 8 males, 1 female) who constituted 3.2% of 281 myasthenic patients attending our department. All had previously received a diagnosis different from myasthenia. Diagnosis of limb-girdle myasthenia was established by clinical, muscle biopsy and electrophysiological assessment including repetitive nerve stimulation and single fiber electromyography. Five patients had the familial form with tubular aggregates in skeletal muscle; four patients had the autoimmune form. Patients with the familial form had a good response to acetylcholinesterase inhibitors, and the patients with the autoimmune form responded to immunotherapy. Our findings reinforce the opportunity to suspect limb-girdle myasthenia in unclassifiable proximal myopathies and to differentiate familial from autoimmune cases, especially for therapeutic implications.

Electrophysiological study of neuromuscular system involvement in mitochondrial cytopathy.

OBJECTIVES: To define the neuromuscular involvement in 'mitochondrial' patients with clinical evidence of a neuromuscular disorder, and to evaluate if the proposed electrophysiological protocol was suitable to reveal a subclinical neuropathy or myopathy in 'mitochondrial' patients with no clinical sign of a neuromuscular disturbance. METHODS: Quantitative concentric needle electromyography (CNEMG), single fiber electromyography (SFEMG) and nerve conduction studies (NCS) were performed in 33 patients with mitochondrial cytopathies. Lastly, we studied 9 clinically unaffected relatives. RESULTS: NCS were abnormal in 18% of patients, with CNEMG and SFEMG in 58% of cases, but there was not a complete overlapping of the positivity of the different techniques. No asymptomatic relatives showed abnormalities of the electrophysiological studies. CONCLUSIONS: Electrophysiological findings did not correlate with any specific biochemical or genetic defect, but were consistent with clinical diagnosis in almost all of the patients with clinical signs of myopathy and/or neuropathy. Increase of both SFEMG jitter and fiber density was significantly tied to a neuropathic process. CNEMG and SFEMG were altered in about 30% of subjects without clinical signs of myopathy or neuropathy and were therefore able to reveal a subclinical involvement of neuromuscular system in some patients who had external ophthalmoplegia or retinitis only.

Focal hand dystonia in a patient with thoracic outlet syndrome.

A patient affected by thoracic outlet syndrome, with an involvement of the left lower primary trunk due to a rudimentary cervical rib, developed a severe hand dystonia on the same side. The dystonic posture was characterised by a flexion of the wrist with the fingers curled into the palm. Polygraphic recordings performed on the left flexor digitorum superficialis (FDS4) and extensor digitorum superficialis (EDC4) muscles, during a repetitive tapping task of the fourth digit, showed a loss of well formed bursts without a clear silent period along with long duration bursts of cocontraction in antagonistic muscles. The study of reciprocal inhibition between forearm flexor and extensor muscles showed a reduced amount of inhibition in both the disynaptic and the later presynaptic phase of inhibition. The patient underwent an operation with resection of the cervical rib. Twelve hours after the operation the patient experienced a significant improvement of the hand dystonia; the distonia had disappeared completely by two months with a progressive normalisation of reciprocal inhibition.

Electrophysiological studies in mild idiopathic carpal tunnel syndrome.

Many techniques have been reported to improve the diagnosis of carpal tunnel syndrome (CTS), but there is no agreement on the diagnostic yield of these different methods. We used an electrophysiological protocol including the assessment of the orthodromic sensory conduction velocity of the median nerve along the carpal tunnel, comparison of median and ulnar sensory conduction between the ring finger and wrist, short segment incremental median sensory nerve conduction across the carpal tunnel recording from the III digit ('inching test'), the study of the refractory period of transmission (RPT) and calculation of the distoproximal ratio obtained by dividing the nerve conduction velocity in the median nerve between the third digit and the palm and between the palm and wrist in 41 patients with mild CTS (75 symptomatic hands) and in 45 control subjects. The distoproximal ratio calculation was the most sensitive technique (81%), but was also the least specific. The 'inching test', even though less sensitive, had the advantage of localising focal abnormalities of the median nerve along the carpal tunnel. RPT was abnormal in patients with recent symptoms. Combining the different techniques, an overall sensitivity of 92% was reached, 11% higher than the yield of the single best test suggesting that a multimodal approach could be useful. The best procedure for electrodiagnosis of mild CTS was to combine the median/ulnar comparison test with calculation of the disto-proximal ratio.

Botulinum toxin in upper limb spasticity: study of reciprocal inhibition between forearm muscles.

To establish whether botulinum A toxin (BTX-A) acts on modifying reciprocal inhibition between forearm muscles in spasticity, 20 patients with post-stroke upper limb spasticity lasting for more than 1 year were studied. Clinical examination, physiotherapeutic evaluation, standardized video-tape assessment and electrophysiological testing (flexor carpi radialis muscle M and H responses with study of reciprocal inhibition) were performed at baseline and 2 weeks, 1, 2, 3, 4 months after BTX-A treatment. BTX-A induced a significant decrease of tone and an improvement of motility and functional status, with a significant decrease of the M wave and the H reflex. The reduction in both inhibitory phases of reciprocal inhibition did not change after BTX-A treatment differently from that reported in upper limb dystonia. These findings indicate that the efficacy of BTX-A in upper limb spasticity is mainly due to peripheral effects.

Unilateral injection of botulinum toxin in blepharospasm: single fiber electromyography and blink reflex study.

We studied six patients affected with blepharospasm (BSP). We injected botulinum toxin (BTX) around only one eye and saline solution around the other. Clinical rating of BSP was performed. Single fiber electromyography (SFEMG), compound motor action potential (cMAP) at the orbicularis oculi muscle by stimulation of the facial nerve, blink reflex, and blink reflex recovery curve were recorded. All clinical and electrophysiological investigations were carried out before, and 1, 2, and 4 weeks after treatment. Evidence of bilateral clinical benefit was provided. Following therapy, facial cMAP decreased bilaterally and SFEMG revealed statistically significant changes on both sides while the excitability curve of blink reflex remained unmodified. The results confirm that BTX affects merely the neuromuscular junctions. The clinical and neurophysiological effects are present on both sides also for unilateral injection probably because of toxin spreading.

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.

Botulinum toxin therapy: distant effects on neuromuscular transmission and autonomic nervous system.

To evaluate distant effects of botulinum toxin, single fibre electromyography on the extensor digitorum communis muscle and six tests of cardiovascular reflexes were performed in five patients injected with BoTox (Oculinum(R) 20-130 units) for craniocervical dystonia and hemifacial spasm. Patients underwent two sessions of treatment and the second time the dosage was doubled. Botulinum toxin injection induced an increase of mean jitter value above normal limits in all cases. An increase of fibre density was recorded six weeks after the treatment. Cardiovascular reflexes showed mild abnormalities in four patients. The data confirm distant effects of botulinum toxin on neuromuscular transmission and on autonomic function.

Single-fiber electromyography for studying the effect of betamethasone on normal neuromuscular junctions in man.

The effects of steroids on normal end-plate in vivo were evaluated in man by using single-fiber electromyography (SFEMG). SFEMG was performed on voluntarily activated extensor digitorium communis muscle under basal conditions and 1 week later an intramuscular injection of 4 mg betamethasone per day was administered for 7 days. Ten patients with low back pain were examined. Steroid therapy did not induce any statistically significant variation either of the mean jitter value or of the percentage of recordings with abnormally high jitter. The results of this study do not give evidence of a direct effect of steroids on normal neuromuscular junction.