Single fiber electromygraphy (SFEMG) is a special EMG technique to study the microphysiology of the motor unit in humans including the functional status of individual motor end-plates in situ, and is known to be a useful test for the diagnosis of neuromuscular juction disorders. To determine the clinical applicability of SFEMFG in this country, 74 SFEMG datas of 61 patients including 54 of myasthenia gravis have been analyzed. The overall ensitivity of SFEMG in myasthenia gravis was high upto 88% and almost all patients with generalized myasthenia gravis showed significant jittering. But the specificity of SFEMG was relatively low(57%) and SFEMG could not clearly differentiate various types of myasthenia gravis. In my experience, SFEMG is a expensive and time-consuming test and I think, at present, SFEMG might have some limitations in clinical usability in this country by the aspect of costeffectiveness.
Diagnosis ; Electromyography* ; Humans ; Korea* ; Motor Endplate ; Myasthenia Gravis* ; Sensitivity and Specificity

Analyzing HI tests of 110 cases of clinical Japanese Encephalitis in 1982, the following results are obtained. 1. The results of HI test are positive in 39 (35.5%), borderline positive in 19 (9.1%), negative in 14 (12.7%) and undetermined in 47 (43.7%) cases. 2. In 49 cases of positive HI test, 14 cases reveal the positive result on the first HI test requested in 5-27 days after the clinical onset of symptoms, and 35 cases show increasing HI titers on the follow-up studies. There is a tendency of increasing HI titers upto 3-4 weeks of onset and sustaining the value for more than two months. 3. In 35 cases with increasing titers on follow-up study, the highest titer is 1:80 in 5 cases, and the half of HI negative cases maintain that value throughout the course. 4. There is no significant statistical differences in clinical characteristics, laboratory and cerebrospinal fluid studies between the patient group of HI positive or borderline and group of HI negative or undermined, except mean hospital day and incidence of coma and death.
Cerebrospinal Fluid ; Coma ; Encephalitis, Arbovirus* ; Encephalitis, Japanese ; Follow-Up Studies ; Hemagglutination Inhibition Tests* ; Hemagglutination* ; Humans ; Incidence ; Korea*

Nerve conduction study is invaluable in clinical neurology, especially for assessing penpheral neuropathies. Abnormal nerve conduction studies may result not only from peripheral nerve dysfunction itself, but also from other various mechanical, technical, and physiological factors. Recently developed electromechanics and standardization of the techniques minimize the variability of nerve conduction studies but physiological factors such as age, sex, and height are not correctable. There are numerous controversies ooncemulg the posslble effects of various physiological factors on the results of nerve conduction studies, probably, in my personal opinion, due to rather small size of the population obtained in the previous studies. Furthermore, the physiological factors them selves are not independent variables to have effect on nerve conduction studies, but they seem to be interrelated. In this study, the results of nerve conduction study on 639 Korean adults over the age of 20 without any suspicion of neurological deficits were analysed to determine the effect of a single independent physiological factor as well as the combined effects of several physiological factors. The results are as follows:1. The nerve conduction velocities varied according to the segments of the tested nerves. The velocities of the upper extremity and proximal segments were faster than those of the lower extremity and distal segments. 2. Comparing my result with those from other laboratories, the interlaboratory variability of nerve conduction velocity seems to be technical. 3. Physiological factors such as sex, age, and height seem to affect the results of nerve conduction studies independently. But stepwise multiple regression analysis has to be utilized because there are statistically significant interrelations between sex and height and between age and height 4. On the stepwise multiple regression anaiysis of the nerve conduction velocities, the sex differences are recognized over the elbow segment of the ulnar motor nerve, the dis.
Adult ; Elbow ; Humans ; Lower Extremity ; Neural Conduction* ; Neurology ; Peripheral Nerves ; Sex Characteristics ; Upper Extremity

Progressive Supranuclear Palsy (PSP) is a rare degenerative disease showing supranuclear ophthalmoplegia, rseudobulbar palsy and several parkinsonian features. This is , as far as the author's knowledge, the first case in this country. The patient is a 55 year old female with slowly progressive disturbances in walking, swallowing, speech and behaviors. The most characteristic finding is the prominent external ophthalmoplegia, especially vertical and downward gaze, which can be easily overcome by the oculocephalic reflexes (Doll's eye movements). The dyspagia, dysarthria and emotional lability may suggest the pseudobulbar palsy. General hyper-reflexia and Babinski sign are noted. There are several features of parkinsonism, but the rigidity is more severe in the trunk than in the extremities and the posture is rather hyperextended than scooped. There is no tremor. The disease is slowly progressive and the medical treatment for Parkinson's disease is not effective.
Deglutition ; Dysarthria ; Extremities ; Female ; Humans ; Middle Aged ; Ophthalmoplegia ; Paralysis ; Parkinson Disease ; Parkinsonian Disorders ; Posture ; Pseudobulbar Palsy ; Reflex ; Reflex, Babinski ; Supranuclear Palsy, Progressive* ; Tremor ; Walking

No abstract available.
Biopsy*

No abstract available.
Biopsy*

This is a case report of oculocraniosomatic neuromuscular disease with "ragged-red fibers". The patients is 36 years old female who is revealing slowly progressive ptosis, external ophthalmoplegia, general weakness and mild right hemiparesis and hemianesthesia. The EMG findings are suggestive of diffuse myopathic process and the frozen muscle specimen from the left deltoid muscle shows the characteristic "ragged-red fibers" with classical myopathic changes.
Adult ; Deltoid Muscle ; Female ; Humans ; Neuromuscular Diseases* ; Ophthalmoplegia ; Paresis

This is a case report of oculocraniosomatic neuromuscular disease with "ragged-red fibers". The patients is 36 years old female who is revealing slowly progressive ptosis, external ophthalmoplegia, general weakness and mild right hemiparesis and hemianesthesia. The EMG findings are suggestive of diffuse myopathic process and the frozen muscle specimen from the left deltoid muscle shows the characteristic "ragged-red fibers" with classical myopathic changes.
Adult ; Deltoid Muscle ; Female ; Humans ; Neuromuscular Diseases* ; Ophthalmoplegia ; Paresis

Blindness or serious visual impairment is a common and well known sequela of methanol intoxication in man, while other manifestations such as focal neurological deficits and abnormal brain CT scan are reported rarely. We experienced a case of acute methanol intoxication, which showed not only visual impairment but also bulbar symptoms and characteristic bilateral symmetrical hypodensity area in basal ganglia, especially putamen on brain CT scan. We tried 'pulse therapy'-continuouis dripping of methyl prednisolone (Solumedrol 1gm/day) for 5 days-with transient improvement of visual impairment, but bulbar symptoms were stationary. One month later, visual impairment was recurred, and the 'pulse therapy' was tried again but without benefit. On follow-up brain CT scan, the size of previous hypodensity area in basal ganglia was decreased.
Basal Ganglia ; Blindness ; Brain* ; Bulbar Palsy, Progressive* ; Follow-Up Studies ; Methanol* ; Prednisolone ; Putamen ; Tomography, X-Ray Computed* ; Vision Disorders

The Lambert-Eaton myasthenic syndrome (LEMS) is a rare disorder of neuromuscular transmission that is frequently associated with malignancy, particulary small cell carcinoma of the lung. To our knowledge, there was no report of LEMS in Korean listeratures and this might be the first case in this country. The patient is a 55 year old man with muscle weakness and fatigability of proximal lower limbs for 2 months. Bronchogenic carcinoma is confirmed radiologically and pathologically. The electrophysiological studies suppert the clinical diagnosis of LEMS with (1) low amplitude of evoked compound muscle potential to a single supramaximal stimulus on nerve, (2) significant decremental response at low rates of stimulation and (3) marked incremental response at high rates of stimulation.
Carcinoma, Bronchogenic ; Carcinoma, Small Cell ; Diagnosis ; Humans ; Lambert-Eaton Myasthenic Syndrome* ; Lower Extremity ; Lung ; Middle Aged ; Muscle Weakness