BACKGROUND: A medulloblastoma is a primitive neuroepithelial tumor of the cerebellum that occurs in children and metastasizes through the cerebrospinal fluid. It is highly malignant and invasive, and the 5-year survival rate is only 60%. Surgical resection techniques, radiation, and chemotherapy have improved the overall survival but the patients suffer life-long cognitive dysfunctions or endocrine abnormalities as the side effects of treatment. Therefore it is essential to identify prognostic markers to determine the appropriate treatment strategy in order to minimize the side effects. METHODS: This study evaluated the immunohistochemical differentiation and survival rate with synaptophysin, glial fibrillary acidic protein, epithelial membrane antigen, vimentin and primitive neuroepithelial marker nestin of 55 paraffin-embedded medulloblastomas, using a tissue microarray. The expression of survivin, the apoptotic inhibitor, and the survival rate with regard to the proliferation index of Ki-67 were also investigated. RESULTS: The group testing positive to vimentin, a mesenchymal differentiation marker, had a worse prognosis and there was a strong correlation between vimentin expression and nestin expression. Patients with a survivin expression rate >35% had a significantly poorer clinical course and there was a correlation between the survivin expression rate and Ki-67 expression rate. CONCLUSION: In conclusion, vimentin and survivin are negative prognostic markers in medulloblastomas.
Cerebellum ; Cerebrospinal Fluid ; Child ; Drug Therapy ; Glial Fibrillary Acidic Protein ; Humans ; Medulloblastoma* ; Mucin-1 ; Nestin ; Neuroectodermal Tumors, Primitive ; Prognosis ; Survival Rate ; Synaptophysin ; Vimentin*

The variations of the sigmoid sinus were studied in 50 Korean adults (average age 67). The classification of the shape and a measurement of the diameters of the sinus were done and the morpological. 1. The shape was classified into 4 types, and the round type was the most common in both sides. S shaped type was below 10% in both sides. 2. The breadth was regular in 54% of the right and in 70% of the left sinuses. According to the breadth change, the shape was classified into 4 shapes. 3. The average diameter was 8.6mm on the right side and 7.2mm on the left side. 4. Symmetry was present in 16% of all. 5. The right transverse sinus was absent in 1 case (2%) and the superior petrosal sinus did not open into the sigmoid sinus in 2 cases (4%). 6. Petrosqamous sinus was observed in 16%.
Adult* ; Classification ; Colon, Sigmoid* ; Humans

BACKGROUND: The H-reflex has been used to assess the proximal nerve conduction in radiculopathy or peripheral neuropathy. The purpose of this study was to evaluate the clinical usefulness of the H-reflex in the diagnosis of subclinical diabetic polyneuropathy. METHOD: Thirty-four diabetic patients (17 women and 17 men) who had neither motor nor sensory symptoms were selected (mean age 57.6 +/- 12.9 years). The duration of diabetes varied from 0.5 to 24 years (mean 6.2 +/- 5.1 years). None of the patients had any known cause of peripheral neuropathy other than diabetes. Twenty-seven healthy subjects (16 women and 11 men) were evaluated as an age-matched control group. H-reflex studies were performed using Braddom and Johnson's methods. The presence and latencies of the H-reflexes were examined in both legs. RESULTS: Twenty-four of the 34 patients (70.6%) had abnormal H-reflex responses (absent H-reflex in 17, prolonged latency in 7). However, only three out of the 27 control subjects (11.1%) had abnormal H-reflex responses (absent H-reflex in 1, prolonged latency in 2). CONCLUSIONS: Abnormalities in H-reflex studies have often been seen in diabetic subjects without overt neurological symptoms. This study suggests that the H-reflex study may be a useful screening tool in the diagnosis of subclinical diabetic polyneuropathy.
Diabetic Neuropathies* ; Diagnosis ; Female ; H-Reflex* ; Humans ; Leg ; Mass Screening ; Neural Conduction ; Peripheral Nervous System Diseases ; Radiculopathy

Hashimoto's encephalopathy (HE) is a steroid-responsive disorder of persistent or relapsing neurological or neu-ropsychological deficits associated with elevated serum concentrations of antithyroid antibody that frequently presents with myoclonus, seizures, and stroke-like episodes. We report a 55-year-old woman who presented with an altered men-tality associated with generalized tonic-clonic seizure. She was diagnosed with Hashimoto's thyroiditis 3 years prior. Serum antithyroglobulin antibody and antimicrosomal antibody were elevated. Electroencephalography (EEG) showed generalized slowing. Brain magnetic resonance imaging (MRI) with T2-weighted sequence and fluid-attenuated inver-sion- recovery (FLAIR) image revealed a high signal intensity in both mesial temporal areas. Single photon emission computed tomography (SPECT) demonstrated decreased perfusion in multiple areas. With the impression of HE, she was placed on high dose steroid and thyroid hormone replacement therapy which ameliorated her neurological symp-toms. We suggest that HE should be included in the differential diagnosis of decreased mentality, especially when patients with Hashimoto's thyroiditis show altered consciousness, seizure disorder, or cognitive decline.
Brain ; Consciousness ; Diagnosis, Differential ; Electroencephalography ; Epilepsy ; Female ; Hormone Replacement Therapy ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Myoclonus ; Perfusion ; Seizures ; Thyroid Gland ; Thyroiditis ; Tomography, Emission-Computed, Single-Photon

A 59-year-old woman was admitted to our hospital because of occipital headache and multiple cranial nerve dysfunction. The patient had been suffered from chronic otitis media. MRI of the brain showed a thickened and markedly enhanced dura mater in the temporal lobe. The patient underwent a left temporal craniectomy and dural biopsy. Histopathological examination revealed thickened duar amter infiltrated with giant cell, lymphocytic and plasma cell infiltration. The fungus, acid-fast and PAS stains were all negative. After administraion of steroid, cranial nerve disturbances and headache improved. The cause of the hypertrophpic intracranial pachymeningitis in this patient was thought to be related with the chronic otitis media, although the clinical course of the patient was similar to the idiopathic hypertrophic pachymeningitis.
Biopsy ; Brain ; Coloring Agents ; Cranial Nerves ; Dura Mater ; Female ; Fungi ; Giant Cells ; Headache ; Humans ; Magnetic Resonance Imaging ; Meningitis* ; Middle Aged ; Otitis Media* ; Otitis* ; Plasma Cells ; Temporal Lobe

Spinocerebellar ataxia (SCA) type 6 was recently identified as a form of autosomal dominant cerebellar ataxia associated with the small expansion of CAG repeats. The number of CAG repeats varies from 4 to 18 on normal alleles and 21 to 30 on the SCA type 6 chromosome. SCA type 6 is characterized by cerebellar ataxia and dysarthria associated with cerebellar atrophy. Many patients with SCA type 6 have horizontal gaze-evoked nystagmus, and some have a limitation of eye movements on the upward and lateral gaze. A 59-year-old woman without any noticeable family history presented with slowly progressive cerebellar ataxia, dysarthria, and oscillopsia. She had vertical nystagmus and horizontal gaze-evoked nystagmus. Brain MRI revealed a moderate cerebellar atrophy, most prominent in the vermis, with relative sparing of the brain stem. A genomic polymerase chain reaction (PCR) analysis showed 24 CAG repeats at the SCA6 locus compatible with the sporadic SCA type 6.
Alleles ; Atrophy ; Brain ; Brain Stem ; Cerebellar Ataxia ; Dysarthria ; Eye Movements ; Female ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Nystagmus, Pathologic ; Polymerase Chain Reaction ; Spinocerebellar Ataxias*

The impairement of glucose metabolism is frequently associated in hyperthyroidism. The present study was performed to determine the effect of the thyroid hormone excess on insulin sensitivity and on insulin secretory function in vivo. Ten newly diagnosed hyperthyroid patients and fifteen healthy control subjects were subjected to frequently sampled intravenous glucose tolerance tests(FSIGT) after an overnight fast. Insulin sensitivity, represented by the insulin sensitivity index(S_1), was assessed by minimal model analysis of FSIGT data. Insulin secretion was measured by the total area under the insulin curve after glucose load.The results were as follows.1) The K_G values, which represent glucose tolerance, were not different between the hyperthyroid patients and the normals(2.2+-0.3 vs. 2.5+-0.3%/min, p>0.05).2) S_1 was significantly decreased in the hyperthyroid patients in comparison to the normals(7.5+-1.4 vs. 2.6+-0.3X10^-4 min^-1/uU/ml, p<0.05).3) The basal insulin concentration was higher in the hyperthyroid patients than in the normals(8.3+-2.4 vs. 4.6+-0.4 uU/ml, p=0.07). In addition, the insulin secretory response to a glucose load was increased in the hyperthyroid patients as evidenced by the peak plasma insulin level(168.2+-30.4 vs. 89.2+-13.9 uU/ml, p<0.05) and by the total area under the insulin curve(2641.1+-443.2 vs. 1696.7+-204.3 min uU/ml, p<0.05).These results clearly demonstrated that insulin sensitivity was impaired in these newly diagnosed hyperthyroid patients. However, glucose tolerance was maintained by the increased insulin secretion.
Glucose ; Glucose Tolerance Test ; Humans ; Hyperthyroidism ; Insulin Resistance ; Insulin ; Metabolism ; Plasma ; Thyroid Gland

Organophosphate (OP) pesticides are the most common source of human toxicity globally, causing high mortality and morbidity despite the availability of atropine as a specific antidote and oximes to reactivate acetylcholinesterase. The primary toxicity mechanism is inhibition of acetylcholinesterase (AchE), resulting in accumulation of the neurotransmitter, acetylcholine, and abnormal stimulation of acetylcholine receptors. Thus, the symptoms (muscarinic, nicotinic, and central nervous system) result from cholinergic overactivity because of AchE inhibition. OP can also cause rhabdomyolysis, pancreatitis, parotitis, and hepatitis. OP therapy includes decontamination, supportive therapy, and the use of specific antidotes such as atropine and oximes. However, there has been a paucity of controlled trials in humans. Here we evaluated the literature for advances in therapeutic strategies for acute OP poisoning over the last 10 years.
Acetylcholine ; Acetylcholinesterase ; Antidotes ; Atropine ; Decontamination ; Hepatitis ; Humans ; Neurotransmitter Agents ; Oximes ; Pancreatitis ; Parotitis ; Pesticides ; Receptors, Cholinergic ; Rhabdomyolysis

Malignant schwannomas arising from the peripheral nerve sheath of the foot are very rare. We encountered a case of malignant schwannoma of the great toe in a 60-year-old woman with painful swelling and exudative ulceration, and report the findings of MR imaging and review the literature.
Female ; Foot ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neurilemmoma* ; Peripheral Nerves ; Toes* ; Ulcer

BACKGROUND: Recent advances have been made in the treatment of acute stroke, but the effectiveness of the new therapies is highly time dependent. Patients with acute stroke often arrive at the hospital too late to receive the maximum benefit from these new stroke therapies. Efforts to reduce delay time of therapy for acute stroke may be more effective if the factors that delay hospital arrival are identified and targeted for specific intervention. So we studied about reason of delay of hospital presentation in patients with acute stroke. METHODS: The 85 acute stroke patients group who admitted to the Young-dong severance hospital from April to August 1999 were enrolled in this study. We collected clinical data from the medical record, including demographic characteristics, date and time of symptom onset, date and time of presentation to the hospital, medical history, and symptoms at stroke onset. And informants about stroke, method of transportation, the patient's interpretation of the symptoms were interviewed. We defined early arrival as within 3 hours of awareness of symptoms. RESULTS: The 85 patient were interviewed, early arrival were more likely to arrive by ambulance(P<0.001), admit via emergency department(P=0.001), interpret their symptoms as a stroke(P=0.005) and use readings as a informants about stroke(P=0.027) than late arrivals. Also they were younger than late arrivals(P=0.027). Main reason of delay of hospital presentation was because they expect spontaneous improvement(43%), mistake as other disease(23.3%), arrive via other medical institute(20%). CONCLUSION: Late arrivals expected spontaneous improvement, misinterpreted their symptoms as those of other disease and didn't choose proper medical institute for acute management. Considerable education is needed to increase the knowledge about stroke and proper acute management.
Education ; Emergencies ; Humans ; Medical Records ; Reading ; Stroke* ; Transportation