Myotonia is characterized by failure of the voluntary muscles to relax immediately and persistence of contraction following voluntery movement or mechanical and electrical stimulation, Myotonic dystrophy (myotonia dystrophica, myotonia atrophica, Steinert's disease) is a hereditary progressive muscular abnormality with dominent transmittance and is characterized by myotonia, muscular wasting and weakness, and frequent association of frontal baldness, testicular atrophy, cataracts, and other nonmuscular abnormalities, We report a case of myotonic dystrophy in a 38 year-old man who had myotonia, muscular wasting and weakness, testicular atrophy, and frontal baldness, Electrodiagnostic studies indicated typical myotonic dystrophy. A review of literature is included.
Alopecia ; Atrophy ; Cataract ; Electric Stimulation ; Muscle, Skeletal ; Myotonia ; Myotonic Dystrophy

Thirty cases of peripheral nerve injury were selected and analysed by means of electromyographic studies at Severance Hospital, Yonsei University, from January 1972 to August 1972. 1. The sex ratio was 17:13 (male: female). The peak incidence occurred in the twenty to thirty year age group. Involved side: right side 18 cases: left side 12 cases. 2. Nerve involved: peroneal nerve (15 cases), tibial nerve (2 cases), median nerve (7 cases), ulnar nerve (4 cases) and radial nerve (2 case). 3. Mode of nerve injuries 1) Peroneal nerve: Nerve compression, variable (10 cases) Tibia & fibular fracture complication (4 cases) Stab wound, fibular neck region (1 case) 2) Tibial nerve: Injection neuritis, buttock (2 cases) 3) Median nerve: Cut glass laceration, wrist region (3 cases) Carpal tunnel syndrome (3 cases) Undetermined mode (1 case) 4) Ulnar nerve: Cut glass laceration, wrst region (2 cases) Forearm bones fracture complication (1 case) Supracondylar fracture (cubitus valgus) (1 case) 5) Radial nerve: Cut glass laceration, wrist region (1 case) Crushing injury, elbow region (1 case) 4. Among the 15 cases of peroneal nerve injury, there were 10 cases of partial denervation and 5 cases of complete denervation. 5. The mode of injury in 10 cases of partial denervation was nerve compression from the following causes: lithotomy posture during forceps delivery (1 case), external rotation of legs during recovery state after surgery (3 cases), abnormal posture during coma state after CO intoxication (2 cases), tight long leg cast (3 cases), and direct trauma while descending stairs (1 case). 6. In all 10 cases of partial denervation of the peroneal nerve, decreased motor nerve conduction velocities, diminished amplitude and prolonged latencies were observed. 7. In partial denervation of the peroneal nerve, the earliest that reinnervation was observed was within 3 weeks in 3 out of 10 cases. 8. Spontaneous fibrillation was observed 3weeks after injury in all cases except one in which it was observed only 7 days after peroneal nerve injury. 9, The earliest appearance of positive sharp waves among all peroneal nerve injuries was observed 2 weeks after injury. 10. The initial appearance of nascent potentials (polyphasic potentials) in peroneal nerve injuries was observed 3 weeks after injury (1 case), 5 weeks after injury (1 case), and 5 months after injury (1 case). They are all partial denervation cases due to nerve compression. 11. Ten cases of partial denervation of the peroneal nerve were treated with electrical stimulation and drop foot board with good recovery. Five cases of complete denervation of the peroneal nerve were treated with neurorrhaphy (1 case) and short leg bracing (4 cases). 12. The initial appearance, of polyphasic potentials was observed 5 months after injury in one case out of 3 cases of median nerve injury. 13. Three cases of median nerve injury were classified as carpal tunnel syndrome and occurred in women only. Observations included absence of sensory nerve action potentials below the lesion, delayed distal latency at wrist and normal motor nerve conduction velocity. They were treated by division of the deep transverse carpal ligament with good result. 14. The initial appearance of nascent potentials was observed 7 months after injury in one case among 3 cases of ulnar nerve injury. 15. In two cases each of radial and tibial nerve injury, no reinnervation pattern was observed until the six month follow-up study after injury. At that time no muscle contraction could be seen or palpated clinically. 16. We observed normal motor unit potentials in the muscles in the follow-up studies even though muscle contraction could not be seen or palpated clinically. 17. Electromyographic examination at selected intervals made accurate diagnosis and prognosis possible and aided in evaluating the course of nerve regeneration, which permictted the choice of appropriate treatment.
Action Potentials ; Braces ; Buttocks ; Carpal Tunnel Syndrome ; Coma ; Denervation ; Diagnosis ; Elbow ; Electric Stimulation ; Extremities ; Female ; Follow-Up Studies ; Foot ; Forearm ; Glass ; Humans ; Incidence ; Lacerations ; Leg ; Ligaments ; Median Nerve ; Muscle Contraction ; Muscles ; Neck ; Nerve Regeneration ; Neural Conduction ; Neuritis ; Peripheral Nerve Injuries ; Peripheral Nerves ; Peroneal Nerve ; Posture ; Prognosis ; Radial Nerve ; Sex Ratio ; Surgical Instruments ; Tibia ; Tibial Nerve ; Ulnar Nerve ; Wounds, Stab ; Wrist

Fibrous dysplasia of bone is a condition affecting one or more bones, usually not disabling, of slow progress, and showing a tendency to become arrested. The etiology of fibrous dysplaia is unknown but it is now believed to be a developmental error in which primitive fibrous tissue proliferates within the bony medulIa and encroaches upon the cortex from within, often producing expansion. The process may be localized to a small segment or may involve almost the entire shaft of the bone, and it may be monostotic, monomelic or polyostotic. In the latter form some patients exhibit a remarkable combination of skin pigmentation and endocrine disturbances. This paper reports the one case of polyostic and three cases of monostotic fibrous dysplasia. The disgnosis was made by clinical, radiological and histological findings.
Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Monostotic ; Humans ; Skin Pigmentation

Forty six cases of Legg-Calve-Perthes disease were reviewed clinically and roentgenographically in order to determine a satisfactory method of assessing the prognosis and to correlate the clinical and roentgenographical results. 1. The patients were children 3 to 12 years of age, with the peak at 6 years. The average age of visit (or diagnosis) was 6.7 years and that of onset was 6.4 years. 2. The total number of hips involved were 49, three cases being bilateral (6. 5%). Boys predominated girls by a ratio of 4.7:1 3. End results in children below 6 years of age were better than those in children above 6 years. The end results of treatment with the containment method were better than those with the noncontainment method. 4. The hips with excellent result had an average of 2.5mm. of uncovering of the femoral head at the time of diagnosis. The degree of uncovering increased as the roentgenographical result deteriorated, in the poor hips the average uncovering being 9mm. The average for the normal side was 2mm. The uncovering at onset is an accurate guide to the likely end result. 5. The Catterall assessment was of reliable prognostic value. In the excellent group, the majority of the hips belonged to Groups 1 and 2, In the poor group, there were no hips in Group 1 and increasing number of hips belonged to Groups 2,3 and 4. 6. The head at risk judged by Gage's sign, calcification of lateral epiphysis, lateral subluxation of the femoral head, and presence of a horizontal epiphyseal line indicated poor prognosis. 7. There was a close correlation between the severity of clinical features and the severity of residual roentgenographic findings.
Child ; Clinical Study ; Containment of Biohazards ; Diagnosis ; Epiphyses ; Female ; Head ; Hip ; Humans ; Legg-Calve-Perthes Disease ; Methods ; Prognosis