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*

In this article, I review various causes of exogenous myoglobinuria(MU) and its pathogenesis in 26 consecutive patients admitted to emergency room, Asan Medical Center and determine whether there is a relationship between concentration of urine myoglobin(Mb) and acute renal failure(ARF) as a complication of MU. Serum and urine Mb were measured by RIA using myoglobin kit (Daiichi, Inc., Tokyo, Japan). The most common disorder of MU was septic shock with hypotension, followed by crush syndrome, major arterial occlusion by thormbosis, alcohol intoxication with status epilepticus, intoxication of unidentified snake venom and drug ingestion. On the basis of this limited amount of data, there is a significant association between high concentration in urine Mb(> 300 ng/ml) and ARF(Fisher's exact test, p< 0.005). To minimize the chances of development of ARF, routine urine Mb levels should be checked on patients at risk, especially septic shock with hypotension.
Adult ; Aged ; Female ; Human ; Kidney Failure, Acute/complications ; Male ; Middle Age ; Myoglobinuria/complications/*etiology ; Retrospective Studies

Multiple sclerosis (MS) is the most common demyelinating disease affecting the central nervous system of young adults living in the western world. MS should be strongly suspected when a young adult develops one or more neurological episodes consistent with damage to white matter within the central nervous system (CNS), especially when these affect the optic nerves, brainstem, or spinal cord. The patient with relapses, each of which can be attributed to demyelination in the CNS, requires no investigation prior to establishing the diagnosis of clinically definite MS. For a diagnosis of MS, separate anatomical sites within the CNS must have been affected on different occasions, typically three. MS in Asian populations is characterized by the selective and dominant involvement of the optic nerve and spinal cord with some incidence of brainstem lesions. 35-40% of MS cases in Korea are of this optico-spinal type with or without brainstem lesions. Reported cases of neuromyelitis optica spectrum disease (NMOSD), causing severe optic neuritis (ON) and/or longitudinally extensive transverse myelitis, either monophase or with a relapse-remitting pattern, some of which were diagnosed previously as the optico-spinal form of MS in Asia, have increased annually in Korea with the development of the NMO-IgG or aquaporin4-antibody detecting technique. NMO-IgG detection is very important in the diagnosis of early stage of NMOSD and the differentiation of MS and other demyelinating disease. Many new convenient oral drugs or very potent intravenous monoclonal antibodies for targeting VLA-4, CD20, and CD52 may decrease the annual relapse rate and burden of brain-spinal cord lesionsin MS.
Antibodies, Monoclonal ; Asia ; Asian Continental Ancestry Group ; Brain Stem ; Central Nervous System ; Demyelinating Diseases ; Humans ; Incidence ; Integrin alpha4beta1 ; Korea ; Multiple Sclerosis ; Myelitis, Transverse ; Neuromyelitis Optica ; Optic Nerve ; Optic Neuritis ; Recurrence ; Spinal Cord ; Western World ; Young Adult

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: 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

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

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