We report a familial case of neuronal type of Charcot-Marie-Tooth disease diagnosed by sural nerve biopsy, nerve conduction study, and electromyography.
Biopsy ; Charcot-Marie-Tooth Disease* ; Electromyography ; Neural Conduction ; Neurons* ; Sural Nerve

Abnormalities in the number and function of platelets may contribute to thromboembolic complications in patients with essential thrombocythemia(ET). Physicians often use hydroxyurea which reduces the platelet count for the initial treatment of ET. A 74-year-old man with ET was presented with headache, dysarthria, and right hemiparesis 10 months after discontinuation of hydroxyurea. Initial platelet count was 450x103/microliter and we gave him heparin. However, his platelet count rised upto 1,019x103/microliter within 4 days. He was on hydroxyurea 1.5 g/day and his symptoms improved with decrease of platelet count. We report a case of left MCA(middle cerebral artery) multifocal infarction in ET after discontinuation of hydroxyurea.
Aged ; Cerebral Infarction* ; Dysarthria ; Headache ; Heparin ; Humans ; Hydroxyurea* ; Infarction ; Paresis ; Platelet Count ; Thrombocythemia, Essential*

Polyarteritis nodosa is one of the necrotizing vasculitis inv'olving small and medium sized arteries. Polyarteritis nodosa may affect many organs such as kidney, musculoskeletal system, gastrointestinal tract, skin, heart, and nerve. In Cupps and Fauci's autopsy studies, muscle involvement was found in 39% of the patients with polyarteritis nodosa. Commonly Polyarteritis nodosa may be presented clinically by myalgia. We experienced a case of polyarteritis nodosa presenting as myositis, diagnosed by muscle biopsy and renal angiography, which appear with unusual presentation of joint contracture in lt elbow and muscle crarnping pain rather than cornmonly presented myalgla.
Angiography ; Arteries ; Autopsy ; Biopsy ; Contracture ; Elbow ; Gastrointestinal Tract ; Heart ; Humans ; Joints ; Kidney ; Musculoskeletal System ; Myalgia ; Myositis* ; Polyarteritis Nodosa* ; Skin ; Vasculitis

Extra-adrenal pheochromocytoma accounts for about 10 to 15 percent of all such tumors and the combination of pheochromocytoma and renal artery stenosis is rare. We report an unusual case of extra-adrenal pheochromocytoma causing renal artery stenosis which was developed in a 11-year-old boy and the literatures were reviewed.
Child ; Humans ; Male ; Pheochromocytoma* ; Renal Artery Obstruction* ; Renal Artery*

No abstract available.
Aneurysm ; Carotid Artery, Internal ; Caves ; Fistula

Vertigo mimicking labyrinthine lesions may have resulted from ischemic insult to the inner ear or the vestibular nerve and nucleus in the AICA infarction syndrome. A 56-year-old female was admitted to the emergency room with vertigo and hearing loss in right ear. On neurological examination, she had left beating jerky torsional and horizontal nystagmus with falling and past pointing to right side. Brain magnetic resonance images showed high signal intensity in anterolateral portion of inferior pons on T2- weighted images. Severe right facial palsy of peripheral type developed 24 hours after admission. Audiometry and electronystagmography documented absent auditory and vestibular function on the affected side. We argue that vertigo of the acute infarction in AICA territory can be involved the eight and seventh nerve root entry zoon and mimic labyrinthine lesions
Audiometry ; Brain ; Ear ; Ear, Inner ; Electronystagmography ; Emergency Service, Hospital ; Facial Nerve* ; Facial Paralysis ; Female ; Hearing Loss ; Humans ; Infarction* ; Middle Aged ; Neurologic Examination ; Nystagmus, Pathologic ; Pons ; Vertigo* ; Vestibular Nerve

The authors retrospectively reviewed the operative cases of 28 patients with lower cervical spine injury from August 1991 to July 1996. Data from charts were reviewed the following clinical parameters, e.g.) etiologies, neurologic findings, degrees of recovery, managements including operative treatments, and complications. The lower cervical injury was most common in men in the third decade. The most common cause, mechanism and site of lower cervical injury were motor vehicle accidents, flexion type injury and C5-6 respectively. The head trauma was frequently combined. In most cases operation for stabilization was done around two weeks after the trauma, but for decompression purpose it was done before two weeks. Three patients who had complete neural injuries died due to pneumonia, adult respiratory distress syndrome, and upper gastrointestinal bleeding. A neurological recovery rate was high in incomplete neural injury group. In conclusion, early reduction and decompression of spinal canal in incomplete neural injury group is favorable for good recovery. Careful attention should be paid on the patient with complete injury for the development of serious complications such as upper gastrointestinal bleeding, ARDS and pneumonia.
Craniocerebral Trauma ; Decompression ; Hemorrhage ; Humans ; Male ; Motor Vehicles ; Neurologic Manifestations ; Pneumonia ; Respiratory Distress Syndrome, Adult ; Retrospective Studies ; Spinal Canal ; Spine*

In two patients with chronic corneal ulcer, resistant to conventional therapy, analysis of tear fluid and observation of the corneal state were performed before and after treatment using autologous fibronectin and aprotinin for the purpose of estimating the effect of treatment. The plasmin activity which was revealed before treatment was absent after treatment, and corneal reepithelialization was observed after treatment. We think the combined therapy with autologous fibronectin and aprotinin may be effective for the treatment of therapy-resistant chronic corneal ulcer.
Aprotinin* ; Corneal Ulcer* ; Fibrinolysin ; Fibronectins* ; Humans

In two patients with chronic corneal ulcer, resistant to conventional therapy, analysis of tear fluid and observation of the corneal state were performed before and after treatment using autologous fibronectin and aprotinin for the purpose of estimating the effect of treatment. The plasmin activity which was revealed before treatment was absent after treatment, and corneal reepithelialization was observed after treatment. We think the combined therapy with autologous fibronectin and aprotinin may be effective for the treatment of therapy-resistant chronic corneal ulcer.
Aprotinin* ; Corneal Ulcer* ; Fibrinolysin ; Fibronectins* ; Humans

There has been controversy between compression of the facial nerve in the cerebellopontine recess and focal demyelination of the facial nerve on the pathogenesis of hemifacial spasm(HFS). To know the pathophysiology of HFS, we performed the facial nerve stimulation test and blink reflex test in 15 patients with HFS. And then we analyzed change of electrophysiologic findings after botulinum toxin in jection. We could not find any differences of latency between affected and unaffected side in facial nerve stimulation test(p>0.05). In the blink reflex test, there were prolonged R1 latency(p<0.01) and occurance of late response on the affected side. There has no change after botulinum toxin injection. These results suggest that there is no facial neuropathy and no electrophysiological evidence of dysfunction in the blink reflex system. Also, there may be two pathophysiologic lesions in HFS. compression and focal demyelination of the facial nerve. And we think that the botulinum toxin is no effect on facial nerve conduction itself.
Blinking ; Botulinum Toxins ; Demyelinating Diseases ; Facial Nerve ; Facial Nerve Diseases ; Hemifacial Spasm* ; Humans