No abstract available.
Foot* ; Hand* ; Paresthesia*

Authors report a typical case of congenital fiber type disproportion (CFTD) with unique clinicopathologic characteristics. The patient was a 13-year-old boy who presented with weakness of lower extremities, especially proximal muscle, since his infancy. He has suffered from severe scoliosis which got worse since the age of 12. He showed mild dysarthria, high arched palate, and fish face. All routine laboratory data were within normal limits. EMG findings suggested myopathy. The muscle biopsy revealed fiber type disproportion with type 1 predominance. While most of the type 1 myofibers were atrophic or normal in size, the type 2 fibers showed universal hypertrophy. The difference of mean diameter between the larger and the smaller fibers was 27.9%. The patient's clinicopathologic settings fulfilled the criteria of CFTD.
Adolescent ; Biopsy ; Dysarthria ; Humans ; Hypertrophy ; Lower Extremity ; Male ; Muscular Diseases* ; Myopathies, Structural, Congenital* ; Palate ; Scoliosis

No abstract available.
Anterior Cruciate Ligament* ; Knee*

No abstract available.
Korea* ; Lung Neoplasms* ; Lung*

BACKGROUND: The clinical and laboratory findings of five patients with tonic pupil (TP) and neuropathy were reviewed for the comprehension of pathogenesis of TP in neuropathy. METHODS: Immunological and nerve conduction studies (NCS) were performed in three patients with Sjogren's syndrome (SS), Miller-Fisher syndrome (MFS), and Adie's syndrome. RESULTS: Upon initial examination, there were no definite sicca syndromes in patients of SS, despite intolerable sensory symptoms. The TP in MFS was improved after intravenous immunoglobulin. Of the cranial neu-ropathies, trigeminal sensory neuropathy was frequent finding. Deep tendon reflexes were absent in all five patients.Absent sensory nerve action potentials and prolonged R1 and R2 of the blink reflex were detected in two SS patients with syncope and asymmetric sensory loss. CONCLUSIONS: These findings in SS patients implicated the possibility of a selective lesion at the level of the dorsal root- or trigeminal- or autonomic- ganglions complicating the TP. In view of the sensory ataxia, opthalmoplegia, areflexia, slow and decreased sensory NCS in the extremity and prolonged R1 and R2, a demyelinating process of postganglionic parasympathetic nerves were suspected to be the cause of the tonic pupil in MFS. Adie's syndrome along with flushing of the left side of the face and chest after exercise, suggested segmental postganglionic lesions of the sympathetic and parasympathetic peripheral nervous systems. In patients with complicat-ing TP and asymmetric progressive sensory neuropathy, the SS has to be considered even if the patient denies the pres-ence of sicca symptoms at first and SS-A/SS-B autoantibody is negative.
Action Potentials ; Adie Syndrome ; Ataxia ; Autoantibodies ; Blinking ; Comprehension ; Extremities ; Flushing ; Ganglion Cysts ; Humans ; Immunoglobulins ; Miller Fisher Syndrome ; Neural Conduction ; Peripheral Nervous System ; Reflex, Stretch ; Sjogren's Syndrome ; Syncope ; Thorax ; Tonic Pupil*

BACKGROUND: Critical illness polyneuropathy(CIP) is a recognized cause of muscle weakness and failure of weaning from a ventilator during the course of sepsis and multi-organ failure. We experienced seven patients of polyneuropathy associated with critical illness, and reviewed the cases in order to characterize the clinical features of CIP. METHODS: We evaluated seven patients who developed polyneuropathy for the first time during intensive care at the Asan Medical Center from Feb, 1998 to Mar, 1999. RESULTS: CIP occurred usually 2-8 weeks after admission to the intensive care unit. All patients received ventilator care due to severe pulmonary problems, which included pneumonia, ARDS, and empyema. Five of them had sepsis. All patients had quadriparesis prominently in the distal area, muscle atrophy, decreased tendon reflexes, and distal hypoesthesia. Electrophysiological and pathologic studies were compatible with axonal polyneuropathy. Five patients recovered from the underlying critical illness and regained their muscle power with improved findings on follow-up nerve conduction studies. CONCLUSIONS: Critical illness should be considered as a cause of polyneuropathy in severely ill patients, especially if associated with sepsis. After recovery from illness, motor weakness as well as electrophysiological findings improved. Failure of weaning from a ventilator may be more affected by pre-existing cardiopulmonary problems than CIP.
Axons ; Chungcheongnam-do ; Critical Illness* ; Empyema ; Follow-Up Studies ; Humans ; Hypesthesia ; Critical Care ; Intensive Care Units ; Muscle Weakness ; Muscular Atrophy ; Neural Conduction ; Pneumonia ; Polyneuropathies* ; Quadriplegia ; Reflex, Stretch ; Sepsis ; Ventilator Weaning ; Ventilators, Mechanical ; Weaning

BACKGROUND: Critical illness polyneuropathy(CIP) is a recognized cause of muscle weakness and failure of weaning from a ventilator during the course of sepsis and multi-organ failure. We experienced seven patients of polyneuropathy associated with critical illness, and reviewed the cases in order to characterize the clinical features of CIP. METHODS: We evaluated seven patients who developed polyneuropathy for the first time during intensive care at the Asan Medical Center from Feb, 1998 to Mar, 1999. RESULTS: CIP occurred usually 2-8 weeks after admission to the intensive care unit. All patients received ventilator care due to severe pulmonary problems, which included pneumonia, ARDS, and empyema. Five of them had sepsis. All patients had quadriparesis prominently in the distal area, muscle atrophy, decreased tendon reflexes, and distal hypoesthesia. Electrophysiological and pathologic studies were compatible with axonal polyneuropathy. Five patients recovered from the underlying critical illness and regained their muscle power with improved findings on follow-up nerve conduction studies. CONCLUSIONS: Critical illness should be considered as a cause of polyneuropathy in severely ill patients, especially if associated with sepsis. After recovery from illness, motor weakness as well as electrophysiological findings improved. Failure of weaning from a ventilator may be more affected by pre-existing cardiopulmonary problems than CIP.
Axons ; Chungcheongnam-do ; Critical Illness* ; Empyema ; Follow-Up Studies ; Humans ; Hypesthesia ; Critical Care ; Intensive Care Units ; Muscle Weakness ; Muscular Atrophy ; Neural Conduction ; Pneumonia ; Polyneuropathies* ; Quadriplegia ; Reflex, Stretch ; Sepsis ; Ventilator Weaning ; Ventilators, Mechanical ; Weaning

Multiple Sclerosis (MS) is the most common demyelinating disease affecting the central nervous system of young adults living in western countries. The clinical suspicion of demyelination, as a pathological process, is high when a young adult develops one or more neurological episodes indicating a damage to white matter tracts within the central nervous system, especially when the optic nerve, brainstem, or spinal cord is involved. The patient with episodes disseminating in time, each of which can be attributed to demyelination, requires no investigation prior to establishing the diagnosis of clinically definite MS, if the age of clinical presentation falls between 20 and 50 years, if different anatomical sites within the central nervous system have necessarily been affected on separate occasions, and if the clinical manifestation is typical for MS. MS in Asian populations is characterized by selective and dominant involvement of the optic nerve and spinal cords, as well as some incidence of brainstem lesion and symptoms. About 35~40% of cases of MS in Korea are of this optico-spinal type. Optico-spinal MS (OSMS) generally has a higher female-to-male ratio than conventional MS. OSMS is also characterized by frequent relapses, severe disability, few brain lesions on MRI, and a very lower incidence of oligoclonal bands in CSF. Neuromyelitis Optica (NMO) (Devic syndrome), which causes severe optic neuritis (ON) and longitudinally extensive transverse myelits either with a monophase or relapse-remitting pattern, is rare in Korean. NMO- IgG was reported to be helpful for the diagnosis of early-stage NMO and differential diagnoses of MS.
Asian Continental Ancestry Group ; Brain ; Brain Stem ; Central Nervous System ; Demyelinating Diseases ; Diagnosis ; Diagnosis, Differential ; Humans ; Immunoglobulin G ; Incidence ; Korea ; Magnetic Resonance Imaging ; Multiple Sclerosis* ; Neuromyelitis Optica ; Oligoclonal Bands ; Optic Nerve ; Optic Neuritis ; Recurrence ; Spinal Cord ; Young Adult

Radiation-induced neuropathy is a rare but well recognized clinical entity although peripheral nerves are considered to be relatively resistant to irradiation. We report three patients with radiation-induced lumbosacral plexopathy, whose characteristic clinical and electrophysiological features can be summarized as follows: 1) 55-58 year old women who were previously treated with radiotherapy for uterine cervix carcinoma: 2) the latent period is to 10 from 13 years: 3)predominantly motor involvement with slowly progressive paraparesis, asymmetrical onset and worse in distal muscle group: 4) painless at onset, with variable degree of sensorv changes: 5)decreased or absent knee and ankle jerks :6)axonal damage in electrophysiologic study: 7)frequent myokymic discharges. Myokymic discharges occur in bursts at regular rate of 0.1 to 8 Hz. In myokymic bursts with more spikes, interburst interval is longer but interspike Interval is shorter.
Ankle ; Cervix Uteri ; Female ; Humans ; Knee ; Paraparesis ; Peripheral Nerves ; Radiotherapy

BACKGROUND: Organophosphate induced delayed polyneuropathy(OIDP) by ingestion is not common, and the mechanism is not well known. In this study, we present clinical characteristics, electrophysiological findings and pathology of sural nerve in our four cases with OIDP. METHODS: Retrospectively, we reviewed 38 patients diagnosed as organophosphate intoxication at Asan Medical Center from January, 1990 to July, 1998. Among these patients we present four patients with OIDP, who received electrophysiological and pathological studies and discuss similar cases from the literature. RESULTS: OIDP occurred usually 2-4 weeks after exposure. They complained quadriplegia, paresthesia and pain mainly in distal extremities. Two patients had facial diplegia. No definite pyramidal sign was found in all patients. Elelctrophysiological study showed sensorimotor(predominantly motor) axonal polyneuropathy with marked denervation potentials in all tested muscles. Follow-up electrophysiological study after two years showed slightly increased amplitude of sensory nerve or compound motor action potentials with persistent denervation potentials in the distal muscles. Sural nerve biopsy confirmed severe axonal neuropathy with marked decrease of large and small myelinated fibers with myelin ovoids. CONCLUSIONS: OIDP was a cause of severe generalized weakness and paresthesia, decreased sensation in distal extremities after high dose organophosphate ingestion and usually occurred two to four weeks later. The prognosis was poor in patients who had severe weakness of four extremities with facial diplegia at the initial examination.
Action Potentials ; Axons ; Biopsy ; Chungcheongnam-do ; Denervation ; Eating ; Extremities ; Follow-Up Studies ; Humans ; Muscles ; Myelin Sheath ; Organophosphate Poisoning* ; Paresthesia ; Pathology ; Polyneuropathies* ; Prognosis ; Quadriplegia ; Retrospective Studies ; Sensation ; Sural Nerve