This study looks at disease diversity, location of lesions, and progression of neuralgic amyotrophy (NA). Forty patients (28 male and 12 female, age range 15 to 70 years) were clinically examined. Muscle atrophy, weakness, and sensory impairment were assessed. Needle EMG and conduction velocities were performed. Careful clinical, electrophysiological, laboratory, and radiological studies excluded other illness. Twenty-two patients were followed for 2 years. Antecedent fever and upper-respiratory tract infection was seen in 22 cases. Pain of sudden onset was always the initial symptom, followed by weakness, mainly in the proximal muscles of shoulder. The affectation was bilateral in 7 cases. Seven cases had a recurrent form of the disease. Clinical and electrophysiological findings suggest axonal lesions of the peripheral nerves, occurring singly (mononeuritis) or in various combinations (mononeuropathy multiplex). Unusual features, such as VII and XI cranial nerves, phrenic nerve, and lateral antebrachial cutaneous nerve affectation, were found. Follow-up showed good function recovery at variable times, even in 1 case with associated myotonic dystrophy (MD). NA is a well-defined entity, with variable clinical expression and data consistent with mononeuropathy or mononeuropathy multiplex, axonal in type. The overall prognosis is good. The progression in a patient with MD suggests that the capability of muscle fiber membrane to accept regenerating nerve sprouts remains in dystrophic muscles.
SUMMARY Electromyography, motor, sensory and mixed nerve conduction velocity, and H reflex were studied in four patients with ataxia-telangiectasia. The earliest and most striking electrophysiological finding was the reduced amplitude of evoked nerve potentials. In the oldest patient, findings suggestive of spinal atrophy and mild reduction of the motor and sensory nerve conduction velocities were found. Reduced amplitude in the evoked nerve potentials can be observed without clinical evidence of peripheral neuropathy. Electrophysiological abnormalities are more severe in older than in young patients. Sural nerve biopsy in one patient showed mild changes: loss of the largest myelinated fibres and demyelination of some fibres. The ratio between maximum conduction velocity of the sural nerve and the diameter of the largest fibres was in the lower limits of the normal range. The resemblance between electrophysiological abnormalities in Friedreich's ataxia and ataxia-telangiectasia is discussed.
S U M M ARY The normal ratio between the amplitude of the sensory evoked potential (SEP) at the wrist on stimulating digits 1, 2, 3, and 5 was determined in 44 healthy adult subjects. The first digit had the larger amplitude, and the fifth digit the smallest SEP. The amplitude expresses the density of sensory innervation in each finger. The ratio between the amplitude of different fingers varied according to the age of the subject. The amplitude of the SEP from a digit innervated by the median nerve decreased in the elderly more than the SEP amplitude of the digit innervated by the ulnar nerve, probably because of a chronic compression in the carpal tunnel. The changes in the normal amplitude ratio can be applied to the topographic diagnosis of radicular and brachial plexus lesions if a fixed segmental sensory innervation of the hand is accepted. In 44 right handed subjects the amplitude of the sensory evoked potentials at the wrist was significantly larger in the left hand. This asymmetry of sensory innervation between hands could be physiological, and suggests a greater density of sensory innervation in the left hand of right handed subjects.
SUMMARYThe sensory conduction velocity from digit to palm and from palm to wrist was determined in median (digit 3) and ulnar (digit 5) nerves in 47 healthy subjects with age range from 21 to 77 years. The decrement of the sensory conduction as a function of age was more marked in the palm to wrist than in the digit to palm segment. Sensory conduction velocity of the ulnar nerve across the elbow was also studied. Irregularities in the shape of the sensory evoked potential recorded above the cubital sulcus were found in 12.76% of cases, especially in subjects over 50 years of age. These results suggest that aging causes decrement in sensory conduction and changes in the shape of the evoked potentials, especially at points where the nerves are more frequently compressed.
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