Neurologic examination for primary care physicians

No abstract available.

A Case of Chronic Inflammatory Demyelinating Polyneuropathy in Lupas Nephritis / 대한신장학회잡지

Systemic lupus erythematosus(SLE) is a multisys-temic disease. Peripheral neuropathy occurs in about 10% of patients with SLE. Chronic inflammatory demyelinating polyneurpathy has been reported rarely in SLE. We experienced a case of chronic inflammatory polyneuropathy in lupus nephritis. 32-year-old housewife presented to chronic progressive muscle weakness and heavy proteinuria. Kidney biopsy showed compatible with lupus nephritis (WHO Class V, membranous nephropathy). Nerve conduction studies showed reduction in conduction velocity and sural nerve biopsy revealed demyeli-nating polyneuropathy. Steroid therapy led to improvement in clinical symptoms and proteinuria.

Treatment of Common Headaches for Primary Care Physicians

No abstract available.

Prognosis of Isolated Symptomatic Middle Cerebral Artery Stenosis

BACKGROUND: Middle cerebral artery (MCA) stenosis is relatively an uncommon disorder that has not been studied as extensively as extracranial or intracranial internal carotid artery. The long term risk of recurrent stroke or transient ischemic attack (TIA) in patients with isolated symptomatic MCA stenosis remains controversial. Transcranial Doppler sonography (TCD) and magnetic resonance angiography (MRA) are excellent noninvasive tools for a prospective study of MCA stenosis and obstruction. We evaluated the prognosis of isolated symptomatic MCA stenosis prospectively. METHODS: We studied 14 symptomatic patients aged 30-56 years with isolated MCA stenosis who had had TIA or minor stroke. Ten patients had history of two or even more stereotyped TIA or stroke and four patients had a history of single TIA or minor stroke. MCA stenosis was assessed by MRA, TCD and/or conventional angiography (CA) in selected cases at entry. CA and/or MRA showed MCA stenosis in the M1 segment in all patients without the evidence of the widespread atherosclerotic disease. Patients with the potentials of embolic source in the heart or the proximal vessel system were excluded. All patients were treated with antiplatelet agents or anticoagulants. The mean duration of follow-up was 22 months (3-39 months) and TCD was done in 2 to 12 month interval. RESULTS: During follow-up, one of 14 patients had TIAs, the other 13 patients experienced neither subsequent transient attack nor new stroke. TCD demonstrated regression of stenosis in 7 patients, and progression in one patient. TCD findings did not change in 6 patients. CONCLUSIONS: These findings suggest that the prognosis is good for patients with isolated symptomatic MCA stenosis under treatment with antiplatelet agents or anticoagulants.

Clinicoradiologic Study on Benign and Non-benign Pontine Hemorrhage

BACKGROUND: Since prognosis of pontine hemorrhage (PH) is dependent on the initial consciousness level, prediction of outcome is not difficult in patients presenting deeply comatose mentality or mild neurologic deficits without altered consciousness. The outcome of PH accompanied by some degree of altered mentality is, however, so various and cannot be easily predicted. The aim of this study was to analyze the radiologic factors determining the prognosis in this subgroup of PH. We also tried to describe the common clinical and radiologic features in patients with benign clinical course, which have rarely been studied systematically. METHODS: Chiefly based on the initial level of consciousness, 42 patients were classified into benign (BH, n=14), non-benign (NBH, n=19), and fatal (FH, n=9) PH. We retrospectively reviewed their medical records and radiologic data. The modified Rankin score was used for evaluating long-term prognosis. In NBH group, transverse and vertical extension index of hematoma, hemorrhage volume, and presence of extrapontine extension were investigated on CT images. RESULTS: Hemiparesis with or without ocular disturbance was the most common manifestation in BH group (64%) and two patients showed neurologic signs identical to lacunar syndrome. The most common location of hemorrhage was unilateral tegmentum (64%). In NBH group, transverse extension index was significantly greater in the patients with worse prognosis, though vertical extension index and extrapontine extension did not seem to be important in predicting the prognosis. CONCLUSIONS: The clinical features simulating lacunar syndrome are frequently found in BH. The degree of transverse extension in the pons is important in predicting the prognosis of NBH subgroup.

Electro-clinico-pathologic Relations of Epileptogenic Foci in Cavernous Angioma

BACKGROUND AND PURPOSE: Cavernous angiomas are frequently encountered in patients with intractable partial epilepsies. Cavernous angioma can make highly epileptogenic foci and dual pathology. Although it is generally thought that the epileptogenic activity originated in neuronal populations adjacent to the lesion, little is known as to the exact location of the epilepsies on electophysiologic, clinical and pathologic view. We investigated nine intractable epilepsy patients with cavernous angioma regarding relation of EEG, semiology and pathology to verify where are the epileptogenic foci in cavernous angioma. METHODS: We included 9 intractable epilepsy patients with cavernous angioma who had been were undergone video-EEG monitoring. They were aged from 15 to 49 years(average:36.7+15.7)and had cavernous angioma in temporal, frontal lobe, or multiple areas(temporal:7, frontal:1, multiple:1 patients). Four patients had invasive EEG study including subdural and/or depth electrodes. Six patients had undergone epilepsy surgery. We analyzed seizure history, semiology of their seizures, interictal and ictal EEG. To know dual pathology, MRI including hippocampal volumetry, invasive EEG, and pathology were studied. RESULTS: Four patients had multiple auras. Eight patients had complex partial seizures and one had right foot clonic seizure, which were related with the location of cavernous angioma. In scalp EEG, ictal recording showed definite EEG changes, but 3 patients had no definite EEG change in some seizures. In invasive EEG with subdural and/or depth electrodes , interictal spikes were more frequently detected than scalp EEG and ictal EEG revealed not only 3 different ictal onset zones in 3 patients but also EEG seizures without clinical events in 3 patients. Regarding dual pathology, mesial temporal involvement was detected in 2 patients in MRI. Among 6 surgery patients 4 patients including 3 patients with normal hippocampus in MRI had hippocampal or dentate gyral change in pathology. Among 4 patients with invasive ictal EEG, 3 patients including 1 patient with normal hippocampus in MRI and pathology had mesial temporal involvement in ictal onset zones. CONCLUSION: Cavernous angiomas can make multiple epileptogenic foci around themselves and often dual pathology of hippocampus, which can be easily detected by invasive ictal EEG but not by imaging and even by pathology. And the foci can have frequent EEG seizures, which do not make clinical events. Precise localization of epileptogenic foci in cavernous angioma were needed to have good medical and surgical treatments.

Familial Occurrence of Moyamoya Disease: Report of Four Patients in Two Families

Moyamoya disease is an occlusive cerebrovascular disease characterized by stenosis or occlusion of main cerebral arteries. The etiology of moyamoya disease is still unclear, but the frequent familial occurrence suggests that some genetic factors may contribute to its etiology. In Korea, though the moyamoya disease is not infrequent, its familial occurrence has not been reported yet. We report four female patients(in two families) of moyamoya disease with its familial occurrence. Two patients are siblings and the other two are mother and daughter. The ages of symptom onset were thirties in three patients and sixties in one patient. Three patients had intracerebral hemorrhage including intraventricular hemorrhage(recurrent in two). We got cerebral angiography in three patients and magnetic resonance angiography (MRA) in one patient. Full laboratory evaluations were done in three patients, including HLA typing in two patients(mother and daughter). All the patients did not have any hereditary disease or any other disease which are known to cause moyamoya-like changes. Considering the reported familial incidence in Japan, careful search for the family members would reveal much more familial cases in Korea also.

Clinical and electrophysiological studies of subacute combined degeneration

BACKGROUND & OBJECTIVES: Subacute combined degeneration (SCD) is a disease of spinal cord involving the posterior and lateral column due to vitamin B12 deficiency. The clinical and electrophysiologic findings of SCD are various. METHODS: From 1989 to 1996, 7 patients were diagnosed with SCD in our hospitals. The diagnosis was made by the neurologic and laboratory findings and electrophysiolgic studies such as nerve conduction studies(NCS) and evoked potential(EP). RESULT: Four patients received gastrectomy. Two had chronic atrophic, gastritis; one of them was assumed to have food-cobalamin malabsorrtion. The remaining one was a heavy drinker. The mean duration of neurologic symptoms was 35.7 months. The most common initial complaint was paresthesia (in 4) and impairment of cutaneous sensation was the most common neurologic sign At the time of diagnosis, 5 patients had myelopathy with was supported by EP in 3(60%). There were abnormal NCS findings in 5 f 6 patients with peripheral neuropathy. In one patient, there was no symptom and sign compatible with myelopathy but median nerve SEP showed bilateral central conduction delay. No one had visual symptoms but prolongation of P 100 was detected in 2 patients. Sural nerve biopsy was done in 2 case, which revealed chronic nonspecific neuropatby in one and chronic axonopathy in the other. Megaloblastic anemia was found in 4 cases and improved by cobalamin therapy in all the parents, in which the follow up hematologic data were available. There as a tendency that nonanemic patients had more severe neurologic symptoms than anemic ones. We could not find any relationship between the duration and severity of neurologic menifestations was best in the patients with the shortest duration of neurologic manifestations and hematologic feature festations. CONCLUSIONS: The authors suggest that early detection and treatment is very important for the improvement of symptoms in SCD.

Dissection of the vertebrobasilar artery

In spite of relatively common references as differential diagnosis in the cases of vertebrobasilar ischemia or infartion, there are only a few reports about dissections of the vertebrobasilar artery(VBA) in Korea. We reviewed medical records and radiographic findings of the 10 patients diagnosed as having dissections of the VBA at Seoul National University Hospital and Seoul City Borame Hospitall since 1994. The 10 patients, all men ranging from 15 to 58 years, did not have the usual risk factors for stroke. In 6 cases, temporally related trauma or exercise was noted. There were also 2 cases of delayed neurologic manifestations from preceeding trauma, developed 74 days and about I year later respectively. Most subjects(9 cases) showed the ischemic symptoms of posterior circulations. Subarachnold hemorrhage was manifested in 2 cases. Magnetic resonance imaging(MRD, magnetic resonance angiography(MRA) and transfemoral cerebral angiography(TFCA) showed irregular narrowing of proximal vertebral artery(VA) with non-visualization of its distal part, thrombosed VA, intramural high signal intensity in VBA, double lumen appearance or fusiform aneurysm. Until now(mean follow up period; 15 months), them are no recurrences with anticoagulation or antiplatelet therapy in the cases of vertebrobasilar ischemia. Dissections of the VBA should be included in the differential diagnosis of vertebrobasilar ischemia or infarction, especially in the young population or in the subjects without common risk factors. The diagnosis can be made on the bases of clinical features and the characteristic findings of MRI, MRA and TFCA.

Optic Neuropathy Associated with Mitochondrial tRNA(Leu(UUR)) A->G(3243) Mutation

Mitochondrial tRNA(Leu(UUR)) A->G(3243) mutation accounts for 80% of all patients with MELAS(mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes), diabetes mellitus with deafness and progressive external ophthalmoplegia. Optic neuropathy has rarely been reported to be associated with the mutation. We found optic neuropathy in two patients with the mutation. To our knowledge, this is the first case report of optic neuropathy associated with the mutation in Korea.

TEE Detection of Aortic Atheroma in Patients with Cerebral Infarction

BACKGROUND: The atheroma in aorta has been emphasized as a potential source of cerebral embolism after the wide use of TEE. But in Korea there has been no report on the frequency of the aortic atheroma in patients with cerbral infarctions. METHODS: Using transesophageal echocardiography, we evaluated aortic atheroma in the thoracic aorta of 44 patients with cerbral infarctions. The aortic atheroma was defined as a raised lesion with an irregular surface of echo shadow. RESULTS: The aortic atheromas were detected in 7 patients(16%) out of 44 patients with cerebral infarction. However, it were detected in only 3 patients out of 71 patients without cerebral infarction. The size of the lesions reaged from 6mm to 16mm(mean 10+/-3.5) and mobility were seen in 2 patients. The lesions were distributed throughout the thoracic aorta. CONCLUSION: Aortic atheroma is a potential source of cerebral infarction. TEE is a useful procedure for the evaluation of the patients with an undetermined cause of cerebral infarction.

Clinical and Electrophysiological Characteristics of Alcoholic Neuropathy

Authors analyzed clinical and electrophysiological features of 35 patients with alcoholic neuropathy who had visited the Boramae City Hospital. All patients were men. Most of the cases drank alcohol everyday. The duration of alcohol consumption is from 2 to 47 years (mean, 17.8 years). Patients with relatively short history of alcohol consumption had a tendency to take more inadequate meals. Clinically we found two distinct separable categories ; one was sensorimotor peripheral neuropathy associated with other alcoholic complications, such as Wemicke and Pellagra disease, delirium tremens, liver disease etc(24 patients). The other had subacutely progressing prominent weakness with sensory change, especially in lower extremities(ll patients). Electrophysiological studies showed decreased amplitudes of sensory or motor compound action potentials with relative preservation of conduction velocity favoring axonal neuropathy. Among the electrophysiological parameters, abnormalities in F-wave(78.3%), H-reflex(86.4%) and sural nerve conduction studies(78.8%) were more frequently observed than in others, which were thought to be more sensitive parameters with early involvement. In terms of electrophysiological pattern there were no definite diffemeces between the two groups.

Cognitive Function in Progressive Supranuclear Palsy:Correlation with Regional Cerebral Perfusion

Although cognitive impairment is commonly included in the clinical manifestation of PSP, the precise nature of these deficits, as well as their anatomical substrates are not clarified. Our, preliminary study and those of others using 99mTc HMPAO brain SPECT or PET showed the derangement of cortical metabolism or blood flow predominantly in frontal area despite the lack of cortical histopathology. To assess the severity and the specific pattern of cognitive impairment in progressive supranuclear palsy(PSP) and its correlation with the regional cerebral perfusion, a neuropsychological battery encompassing measures of intelligence, language functioning, verbal/visual memory, attention, psychomotor speed and dexterity, visuospatial construction, mental flexibility, and concept formation and reasoning ability was administered to patients with PSP and age/sex matched controls. Patients with PSP had significantly diminished K-WAIS IQ scores when compared with control subjects, although the patients still had "average level' IQ scores. And the patients were particularly impaired when a task required executive and attentional procedures such as abstract concept formation, reasoning, and conceptual shifting. In addition, the severity of frontal lobe dysfunction was well correlated with the regional perfusion index of superior frontal cortex. These results suggest the frontal lobe dysfunction may be the relatively specific aspect of cognitive impairment in PSP and well correlates with the regional cerebral perfusion pattern of PSP.

Clinical Analysis of 26 Patients with Alcoholic Pellagra Encephalopathy

We analyzed 26 cases of probable alcoholic pellagra encephalopathy (APE) in the chronic heavy alcoholics, associated in 18 cases (69%( with Wernicke's disease and in 15 cases (58%) with alcoholic peripheral neuropathy. They were all males and their average age was 46 years. Daily average amount of drinking was 220gm and average period of drinking was 21.8 years. Clinical manifestations were dermatitis in 23 cases (88%), dementia in 22 (85%) and diarrhea in 12 (46%), but only 7 case (27%) had full triad. Most striking features of the APE were fluctuating confusion and/or clouding of consciousness, marked oppositional hypertonus, myoclonic jerks, severe muscle guarding or tenderness in the abdomen and long-lasting (2 weeks or more) delirious behavior. Twelve patients died of sepsis or pneumonia (3 untreated, 9 of 23 treated with nicotinamide). In conclusion, APE seems to be a fatal disease if untreated. Therefore, even in absence of dermatitis or diarrhea, APE should be considered in heavy chronic alcoholics who develop the deterioration of mentality, associated with above-mentioned clinical features, and they should be treated with multivitamins including nicotinamide.

Prevalence of the patent foramen ovale in young patients with ischemic cerebrovascular disease: Transesophageal contrast echocardiographic study

BACKGROUND: A paradoxical embolism through the patent foramen ovale has been suggested as a possible cause of ischemic cerebrovascular disease in young patients without other cardiovascular risk factors, however, the transesophageal contrast echocardiographic examination is proved to be sensitive and accurate to detect the patent foramen ovale in vivo by demonstration a right-to-left shunting of microbubbles at the interatrial septum. METHODS: Transesophageal contrast echocardiographic examinations were performed in 16 young patients(32+/-6 years, 19~39) with ischemic cerebrovascular diseases without other cardiovascular risk factors. Contrast agents were injected twice in each patients, one with Valsalva maneuver and the other with coughing and the presence of the patent foramen ovale was confirmed by demonstrating echogenic contrast crossing the interatrial septum. RESULTS: The patent foramen ovale was demonstrated in five of sixteen patients(31.2%) during transesophageal contrast echocardiographic examination. Although the prevalence of the patent foramen ovale in normal population has not been examined in this study, the prevalence in patient group appears to be significantly higher than that of normal population. CONCLUSIONS: Taken together, a paradoxical embolism through the patent foramen oval appears to be one of the causative factors and a transesophageal contrast echocardiography is recommended especially in young ischemic cerebrovascular disease patients who have normal transesophageal echocardiographic findings and no known risk factors.

Detection of Brainstem Lesion in Wilson's Disease using Brainstem Auditory Evoke Potential Study

No abstract available.

Clinical Observations on Cerebral Sparganosis

We have met 5 cases of cerebral sparganosis since 1984, including one which was reported by Hong et al. In 1985. We reviewed their medical records and clinical manifestations, possible etiologic histories, findings of brain CT and micro-ELISA done in all cases, and findings of brain biopsy done in 2 cases were discussed. 1. Initial manifestations were seizures (in 4 of 5) or limb weak-nesses (in 4 of 5), which were followed by symptoms and signs due to space occupying effects. 2. Raw snakes or raw frogs were ingested in 3 cases and estimated incubation periods in 2 cases were 2 and 5 years respectively. 3. Brain CT's showed widespread low density, usually in white matter area, ipsilateral ventricular dilatation and/or enhaneing nodules. 4. Micro-EILSA's showed that initial titers of sparganum-specific Ab were above criterion (0.22) except for serum of case 5, and F/U micro-ELISA'S done in 3 cases revealed negative conversion (decrease of Ab titer below criterion) only for CSF of case 5, in which surgical removal was successfully done, and otherwise variable changes of titers. 5. Brain biopsies done in 2 cases showed such findin-gs as degenerating worm, granuloma, tunnel formation, and calcospherules.

Chronic Polyneuropathy with Monoclonal Gammopathy of Undetermined Significance

Diseases of the peripheral nervous system are one of the most difficult subjects in neurology partly in that specific etiologies are often not found. Among peripheral neuropathies of unknown causes, an association of monoclonal gammopathies has been observed. Monoclonal gammopathies are disorders characterized by proliferation of a single clone of plasma cell that produces monoclonal proteins. Here we report two cases of chronic sensorimotor polyneuropathy with monoclonal gummopathy of undetermined significance, who showed no evidences of multiple myeloma, macroglobulinemia, systemic amyloidosis, or other neoplasms. One case is also associated with hyperthyroidism and cryoglobulinemia.