[Symptoms and clinical course in carpal tunnel syndrome]. / Symptomer og kliniske forløp ved karpaltunnelsyndrom.

BACKGROUND: We wanted to study the symptoms in carpal tunnel syndrome, evaluate postsurgical median nerve recovery in elderly patients compared to younger patients, and investigate the clinical development and median nerve changes in non-operated cases. MATERIAL AND METHODS: The study included 92 patients diagnosed with carpal tunnel syndrome on the basis of clinical symptoms and neurophysiological findings. Neurophysiological data from elderly operated (70-89 years), younger operated (30-69 years) and non-operated (25-83 years) patients were analysed separately. RESULTS: The most common symptoms were paresthesia in the third (97%) and second (88%) digits, nocturnal paresthesia (88%), increased symptoms when at work (78%), and pain in the hand (72%). Paresthesia in the entire hand (30%) and pain in the entire arm (55%) were common. Median nerve recovery in the elderly patients was comparable to the results in the younger operated patients. Some patients recovered clinically with normalisation of the median nerve without surgery. INTERPRETATION: Carpal tunnel syndrome will often have symptoms outside the median nerve territory. Elderly patients should be operated on the same indications as those applied to younger patients. Patients with no neurological findings may be monitored without surgery.

Median nerve recovery in carpal tunnel syndrome.

We studied recovery of median nerve function in operated elderly and younger patients with carpal tunnel syndrome, compared to nonoperated patients, using neurophysiological methods. Three groups were analyzed separately: elderly operated (n = 16, aged 70 to 89); younger operated (n = 83, aged 30 to 69); and nonoperated (n = 52, aged 25 to 83). The elderly operated patients had significant improvement after surgery in all sensory nerve variables and a decrease in motor distal latency. The results were comparable to the results in the younger operated patients. In the nonoperated hands, the same neurophysiological variables were also significantly improved, but to a lesser extent than in the operated hands. Overall symptom relief was better in the operated hands.

[Congenital myotonic dystrophy]. / Dystrophia myotonica congenita.

Congenital myotonic dystrophy is a distinct entity causing severe hypotonia and other neuromuscular manifestations. The disease is usually inherited from the mother and shows a more severe course than the adult form of dystrophia myotonica. Congenital myotonic dystrophy is associated with increased perinatal mortality. We describe a case with this disorder and discuss the differential diagnosis in relation to other neuromuscular disorders causing hypotonia in early life.

Hereditary essential myoclonus.

A Scandinavian family whose members have suffered from hereditary essential myoclonus for five generations are presented. Three patients showed a uniform clinical picture with myoclonic jerks located in the upper trunk, the neck and proximal parts of the upper extremities without any other symptom or sign of CNS-dysfunction. The laboratory findings including EEG and somatosensory evoked potentials (SEP) were normal. Diagnostic criteria are discussed. Treatment was relatively ineffective.

Beneficial effects of plasma exchange in acute inflammatory polyradiculoneuropathy.

The results of a controlled trial in which 38 patients with severe acute inflammatory polyradiculoneuropathy took part indicate that plasma exchange favourably influenced the course of the disease. Significant benefits were seen in time until onset of improvement, course of muscular weakness, improvement in disability grades over the first 2 months, and working capacity after 1 month. Cost-benefit analysis showed that the exchange treatment resulted in net financial savings. The results suggest that plasma exchange may have a role in the treatment of severe acute inflammatory polyradiculoneuropathy.

Pathophysiology of fasciculations in ALS as studied by electromyography of single motor units.

Electromyographic potentials of fasciculations were studied in ten patients with amyotrophic lateral sclerosis (ALS). The EMG recordings were made from the extensor digitorum brevis muscle. The EMG recording was so selective that only one motor unit potential appeared on maximal voluntary effort and on supramaximal electrical stimulation of the peroneal nerve. In a series of fasciculations, the shapes of the EMG potentials varied, while in a series of voluntary twitch activations of electrical nerve stimulations the EMG potentials were mainly constant. Fasciculations were followed by antidromic impulses in the test unit axon as judged from collision tests, and they persisted after lidocaine blockades of the nerve to the muscle. The findings are compatible with a conclusion of distal multifocal triggering of fasciculation. Fasciculating motor units had voluntary firing properties close to those of normal low-threshold motor units. Widespread fasciculations were abolished by a nonparalytic dose of a synthetic curare derivative (Pavulon) and augmented by administration of neostigmine in two cases. The fasciculations in ALS thus have the same characteristics as experimental fasciculations evoked by cholinesterase inhibitors, and there is reason to believe that the underlying pathophysiological mechanism is similar in the two cases.