Trigeminal motor neuropathy is a rare disorder which involves only motor fiber in trigeminal nerve. We experienced a 58-year-old man with muscle weakness and wasting of left masticatory muscles for 3 weeks. He had suffered from the same event on the right side 13 years ago. He had no sensory dysfunction and Brain CT revealed no gross abnormality. All the laboratory findings were negative and the EMG revealed chronic denervating process in bilateral masseter and temporalis muscles. He has improved after steroid therapy.
Brain ; Humans ; Masticatory Muscles ; Middle Aged ; Muscle Weakness ; Muscles ; Trigeminal Nerve

High dose intravenous methyl prednisolone was administered to patients with multiple sclerosis (4), transverse myelitis (6), and arachnoiditis (I). Almost complete remission was noted in 5 cases (4 with transverse myelitis and one with multiple sclerosis) and partial improvement in 3 patients (2 with multiple sclerosis and I with arachnoiditis ) within 3 days after therapy. The benefits of this therapy, however, was not that dramatic when started late after the onset of neurologic deficits.
Arachnoid ; Arachnoiditis ; Humans ; Multiple Sclerosis ; Myelitis, Transverse ; Neurologic Manifestations ; Prednisolone

BACKGROUND: Neck flexion risks endobroncheal intubation when the tracheal tube is not in the proper position. So accurate knowledge of upper airway length is required to prevent malpositioning of endotracheal tubes. Therefore we evaluated the length of various portions of upper airway in Korean adults (n=500) who had no abnormality of upper airway. METHODS: Five hundred patients, composed of 198 males and 302 females who underwent elective surgery, were included in this study. After endotracheal tube was placed under general anesthesia, we measured the distance from tube machine-end to upper incisor (value 1), from tube machine-end to inferior margin of cricothyroid membrane (value 2), and from tube machine-end to carina (value 3) by means of fiberoptic bronchoscopy. RESULTS: The mean length between upper incisor and inferior margin of cricothyroid membrane (value 2 - value 1) was 12.7 cm in males and 11.6 cm in females, while the mean length between superior margin of cricoid cartilage and carina (value 3 - value 2) was 12.9 cm in males and 11.3 cm in females. So the mean length between upper incisor and the mid portion of trachea was nearly 19.6 cm in males and 17.7 cm in females. CONCLUSIONS: We believe that, based on the findings in this study, the safety length for endotracheal tube fixation is 20 cm in Korean adult males and 18 cm in Korean adult females.
Adult* ; Anesthesia, General ; Bronchoscopy* ; Cricoid Cartilage ; Female ; Humans ; Incisor ; Intubation ; Male ; Membranes ; Neck ; Trachea*

Isolated angiitis of the central nervous system is characterized by necrotizing vascular inflammation confined to the brain or spinal cord. The present report described a 22 year old female patient who experienced abruptly developing headache, dysarthria, swallowing difficulty, quadriparesis, and mental changes. The laboratory test of her blood, urine, and cerebrospinal fluid are all within normal range. Bliateral multiple small areas of low density were noted in the deep brain, cerebellun, and brainstem on brain C-T scan. There is no specific abnormal finding on the cerebral angiogram. The steroid treatment, 3 days of the 'pulse theraphy' followed by oral administration of prednisolone, dramatically ameliorated almost all symptoms and signs at first, but her symptoms were relapsed during tapering of oral prednisolone soon after. The second trial of the steroid treatment was not effective but there was a good response to the combined treatment of cyclophosphamide (Cytoxan) and prednisolone. The repeated brainstem auditary evoked potentials (BAEP) and somatosensory evoked potentials (SSEP) were helpful to determine the clinical course, as well as fluctuating abnormalities on brain CT scans.
Administration, Oral ; Brain ; Brain Stem ; Central Nervous System* ; Cerebrospinal Fluid ; Cyclophosphamide ; Deglutition ; Dysarthria ; Evoked Potentials ; Evoked Potentials, Somatosensory ; Female ; Headache ; Humans ; Inflammation ; Prednisolone ; Quadriplegia ; Reference Values ; Spinal Cord ; Tomography, X-Ray Computed ; Vasculitis* ; Young Adult

Painful ophthalmoplegia is not common and may be due to variety of causes, of which distant metastsis of malignant lymphoma is rare. This is a case of painful ophthalmoplegia who has looked like Tolosa-Hunt syndrome at first showing normal CSF findings, increased erythrocyte sedimentation rate and some response to steroid. About 2 months later, however, pelvic malignant lymphoma (diffuse undifferentiated according to Rappaport classification) was found, followed by lymphomatous meningitis. The symptoms of pain and ophthalmoplegia were markedly improved after systemic and intrathecal chemotherapy and radiotherapy of whole brain for the metastatic malignant lymphoma.
Blood Sedimentation ; Brain ; Drug Therapy ; Lymphoma* ; Meningitis ; Ophthalmoplegia* ; Radiotherapy ; Tolosa-Hunt Syndrome

Vincristine has been extensively need in chemotherapy to treat leukemia, lymphoma, and a variety of solid tumor. The major antitumor effect has been known to be associated with its high affinity binding to the basic protein sub unit of microtubule, tubulin, which causes disruption of the mitotic spindle apparatus and arrest of calls in metaphase. The principal side effect that has commonly limited the use of this agent is peripheral sensori-motor neuropathy, a feature probably related to the disruption of microtubule in neural tissues. There has been a need for animal experimental models w study the peripheral neuropathy induced by vincristine. However, it was not easy to develop an adequate model due to reported interspecies difference of susceptibility to vincristine. In addition, electrophysiologic test methods to confirm peripheral neuropathy in small experimental animals has not been properly addressed. The purpose of this experiment is to find out whether rat can be used as an animal experimental model of vincristine neuropathy. The authors also incorporated serial noninvasive electrophysiololgic tests, in attempt to correlate morphologic alterations induced by vincristine and functional status of peripheral nerve and muscle. Experiment Group 1 of 14 rats were given 0. 2mg/kg vincristine sulfate once a week, Group 2 of 14 rats were given same does twice a week and Group 3 of 14 rats were given 0. 4mg/kg intravenously, through tail veins for 6 weeks, to delineate the possible different effects from dosage and frequency of injections. The 14 rats for control were given only normal saline with the same methods. The electrophysiologic tests including motor nerve conduction study, sensory nerve conduction study and cervical somatosensory evoked potential were per formed 2 weeks interval. The morphologic examinations of posterior tibial nerves using light microscope and electron microscope were done 4 weeks interval up to 16 weeks. The H&E, modified Gomori-trichrome and histochemical stain(ATPase & NADH) 1. Vincristine induced peripheral neuropathy was successfully established in rat.. This was confirmed not only by morphologic measurements but also by noninvasive serial electrophysiologic examinations of peripheral nerves. 2. The vincristine neuropathy in rats was sensori-motor type similar to those in human. 3. The motor and sensory conduction velocity of posterior tibial nerve in rats fell significantly at ter 2-4 weeks in the vincristine injected groups A tendency of recovery was noted, but the conduction velocity failed to return to normal level up to 16 weeks follow up study. However, the amplitude of compound muscle action potentials as well as compound nerve action potentials showed a great deal of fluctuation during the genesis of neuropathy. Therefore, these electrophysiologic parameters were not optimal predictors in assessment of functional integrity of given nerves with the electrophysiologic test methods used in this experiments. 4, Morphologic examinations revealed that vincristine neuropathy I rats are clearly these of axonal degeneration, compatible to the findings of electrophysiologic examination. 5. The higher dose of vincristine induced the more damage to the peripheral nerves but it also resulted in high mortality rate. Administration of 0.2mg/kg/week would be adequate in generation of experimental neuropathy. 6. The examinations of gastrocnemius and soleus muscle showed same evidence of mild degree of myopathy but it was felt to be direct toxic effect, rather than secondary changes due to the neuropathy.
Action Potentials ; Animal Experimentation ; Animals ; Axons ; Drug Therapy ; Evoked Potentials, Somatosensory ; Follow-Up Studies ; Humans ; Leukemia ; Lymphoma ; Metaphase ; Microtubules ; Mortality ; Muscle, Skeletal ; Muscular Diseases ; Neural Conduction ; Peripheral Nerves* ; Peripheral Nervous System Diseases ; Rats* ; Spindle Apparatus ; Tibial Nerve ; Tubulin ; Veins ; Vincristine*

No abstract available.
Animals ; Mice*

Mobius syndrome is a rare congenital disorder characterlzed by facial diplegia and bilateral abducens palsy, which occasionally combinds with other cranial nerve dysfunction, cardiac anomalies, endocrinopathy. Myopathy, peripheral neuropathy and skeletal abnormalities. We report 2 cases of Mobius syndrome; A 6 year old boy showing delayed development, musculo-skeletal anomaly, hypoglossal dysfunction, carpal tunnel syndrome and mild endocrinologic dysfunction, and a 23 year-old lady with only questionable endocrine dysfunction. MRI scans of brain and brainstem are normal and there is no definite electrophysiological evidence of dysfunctions in brainstem on evoked potential blink reflex study. Mental functions look normal in both cases.
Blinking ; Brain ; Brain Stem ; Carpal Tunnel Syndrome ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Cranial Nerves ; Evoked Potentials ; Humans ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome* ; Muscular Diseases ; Paralysis ; Peripheral Nervous System Diseases ; Young Adult

Twenty patients with clinically definite multiple sclerosis were seen at the Department of Neurology, Severance Hospital between 1984 and 1991 and their clinical features ar.d multimodality evoked potential findings were analyzed. We could not find any significant differences in sex ratio, age of onset, and clinical courses from the study results of Japanese and Western groups. However, visual disturbances at the onset and optic-spinal or optic-spinal-brainstem involvements, which are known to be characteristic features of oriental patients, were also noted to be the most common features in our study. Multimodality evoked potential studies were performed and abnorrnal recordings by at least one of the examinations were found in all but one patient. VEP yielded most frequent abnormalities Diagnostic yield of BAEP increased from overall 43% to 83% in cases having brainstem samploms or sings and the increase was much higher than those seen in VEP of SSEP. Increase or increase in latency as occasionall noticed during follow up but majority of patients did not show significant changes in EP {indings. In one patient however. EP abnorrnalities showed improvement in 2 follow up exarninations during 4 years and 7 months despit the evident aggravation in clinical features. Evoked potentials can give valuable information in the diagnosis of multiple scierosis but further studies are necessary to evaluate their usefulness in the follow up examination.
Age of Onset ; Asian Continental Ancestry Group ; Brain Stem ; Diagnosis ; Evoked Potentials* ; Follow-Up Studies ; Humans ; Multiple Sclerosis* ; Neurology ; Sex Ratio

Systemrc lupus erythematosus (SLE) is an autoimmune, inflammatory, chronic disorder characterized by multiorgan involvement including the nervous system. The neurologic manifestations of SLE vary in clinical signs and clinical course. We recently reviewed medical records of 198 patients with SLE who had been admitted to Severance hospital between 1980 and 1990 to see neurological features with the relationship of non-neurological features of SLE and prognosis. Among 198 cases with SLE sixty-seven patients (33.8%) had significant neurologic and/or psychiatric manifestations dunng the course of their disease. CNS lupus was found in 48 cases and symptoms were seizure, mental deterioration, psychiatric manifestations and focal neurologic deficit in decreasing order of frequency. Myelopathy was noted in 4, peripheral neuropathy in 5, and rline patients developed myopathy. Eight had typical meningitis ;four of these patients had aseptic meningitis, three had tuberculous meningitis, and one had bacterial meningitis. In half of the patients with CNS lupus, the neurologic symptoms occurred during the first one year of their illness. Other major organs such as the kidney, heart, lung, skin and joints were more commonly affected in CNS lupus. The prognosis of CNS lupus is relatively poor, especially in patients with seizure which is followed by altered mentality.
Heart ; Humans ; Joints ; Kidney ; Lung ; Lupus Erythematosus, Systemic* ; Medical Records ; Meningitis ; Meningitis, Aseptic ; Meningitis, Bacterial ; Muscular Diseases ; Nervous System ; Neurologic Manifestations* ; Peripheral Nervous System Diseases ; Prognosis ; Seizures ; Skin ; Spinal Cord Diseases ; Tuberculosis, Meningeal