Heavy metal concentrations in hair of newly imported China-origin rhesus macaques (Macaca mulatta) / 한국실험동물학회지

Macaque monkeys are good sentinel to humans for environmental pollutions because their similarities in genetic and physiological characteristics. So, their reference values about exposures to heavy metals are required for proper data interpretation. Here, we report several heavy metals concentrations in the hair of rhesus monkeys which are widely used in biomedical research. The hair of 28 imported rhesus monkeys from an animal farm in southwest China were examined for the presence of eight heavy metals (Arsenic, Beryllium, Cadmium, Chromium, Iron, Lead, Mercury, and Selenium). The analyzed data in parts per million (ppm) for hair concentrations of heavy metals in rhesus monkeys were as follow: As (0.654+/-0.331), Be (0.005+/-0.003), Cd (0.034+/-0.022), Cr (11.329+/-4.259), Fe (87.106+/-30.114), Pb (0.656+/-0.613), Hg (0.916+/-0.619), and Se (3.200+/-0.735). The concentrations of Be, Cr, and As showed significant higher in females than in males (P<0.05). We present here the reference values of several heavy metals in healthy China-origin rhesus monkeys. These data may provide valuable information for veterinarians and investigators using rhesus monkeys in experimental studies.

Clincal, MRI and MRA Findings of Cheiro-Oral-Pedal syndrome

BACKGROUND: Cheiro-oral-pedal syndrome is characterized by a unilateral or bilateral sensory disturbance in mouth corner and hand and/or foot, and associated with central nervous system disease. Causative diseases are cerebral infarction, intracranial hemorrhage, brain tumor, migraine, etc. When related with cerebral infarction, it is known to have lacunar type pathology and favorable prognosis. But other pathogenic mechanism and poor prognosis is suggested. METHODS: We retrospectively studied 12 patients presented with cheiro-oral-pedal syndrome, who visited the department of neurology in Chosun University Hospital from September 2000 to August 2002. Cheiro-oral-pedal syndrome was diagnosed according to the clinical manifestations and brain MRI findings. We assessed neurological findings, brain MRI and MRA findings, duplex sonographic findings, risk factors of stroke and outcome of the patients. Followup period was 3-20 (mean 8) months. RESULTS: There were 12 patients (10 men, 2 women) with the age ranged from 45 to 80 (mean 57.8) years. Sensory loss on cheiro-oral area was present in 8 (66.7%) and on cheiro-oral-pedal area in 4 (33.3%). Responsible lesions were found in thalamus, capsulo-striatum, corona radiata, pons, and fronto-parietal cortex. Three patients had a major cerebral vascular abnormality on MRA. One had MCA stenosis, the other PCA stenosis and another distal ICA stenosis. Sensory symptoms had remained in all patient except one. In-hospital mortality and other neurological deficit at discharge were absent in all. CONCLUSIONS: Cheiro-oral syndrome has mixed pathogenic mechanism of small vessel disease and large vessel atherosclerosis and involves various sites. Paresthesia itself has bad prognosis but overall clinical course is benign.

The Social Stigma of Epilepsy in Korea / 대한간질학회지

BACKGROUND: To study social stigma of people with epilepsy (PWE) in Korea, focusing on which factors are associated with social stigma. METHODS: Data were collected from 400 adult epilepsy patients (51% male, mean age:32.9 years, range:19-64 years). Patients were recruited from 10 epilepsy centers in Korea. Clinical information about seizures was obtained by neurologists and the other information was collected by using self-completed questionnaires including stigma of epilepsy scale and several scales or questions. RESULTS: Thirty one percent of PWE felt stigmatized by epilepsy, 9% of them highly so. The presence of stigma of epilepsy was associated with duration of epilepsy, seizure remission or frequency, generalized tonic clonic seizures in the last 2 years, seizure-related injuries in the last year, the actual discrimination from society, all subscales of QOLIE-31, education, introverted and neurotic personality, helplessness, control, and confidence in problem solving, anxiety and depression, and self-esteem. Multivariate analysis identified discrimination from society, introverted personality, problem solving control, and emotional subscale of QOLIE-31. CONCLUSION: Our data showed the prevalence of social stigma of PWE in Korea might be not high unexpectedly and suggested that the actual discrimination from society and patients' coping style including personality might be important in feeling social stigma of epilepsy. Our results would be basic information for the further studies to clarify the causative factors to generate the stigma of epilepsy.

Magnetic Resonance Angiographic Findings and Clinical Outcome of the Striatocapsular Infarction

BACKGROUND: Striatocapsular infarction involves the territory of the large lateral striate branches of the middle cerebral artery sparing the overlying cortex. Two kinds of angiographic findings of the striatocapsular infarction have been reported previously: one with a complete occlusion of the M1 segment and the other without. However, the clinical features and outcomes in relation to the magnetic resonance angiographic (MRA) findings remain unknown. In the present study, we evaluated the clinical outcomes in patients with striatocapsular infarctions according to the MRA findings. METHODS: Twenty-two consecutive patients with striatocapsular infarctions who were admitted to the Chosun University Hospital between July 1998 and June 2000 were included in the study. Striatocapsular infarction was confirmed by magnetic resonance imaging (MRI) and MRA. The patients were divided into two groups based on MRA findings. Ten patients with occluded MCA were included in group A, and the remaining 12 patients with normal or stenotic MCA were categorized as group B. We compared their clinical characteristics and outcomes at 3-months post-stroke. RESULTS: Only 1 out of 12 patients in group B and 6 out of 10 patients in group A showed cortical symptoms and signs. Patients in group A tended to have more severe disability and a worse prognosis than those in group B. CONCLUSIONS: The MRA findings may help clinicians to better predict the clinical outcomes after striatocapsular infarctions. (J Korean Neurol Assoc 19(5):452~456, 2001)

Four Cases of Paramedian Thalamopeduncular Artery Infarction

Cerebral infarction in the territory of the paramedian thalamopeduncular artery (PTA) causes various lesions in the upper midbrain and thalamus resulting in widespread disturbances in neurological function. However, the exact topography and variations in the territories of the PTA remain unknown. We report four patients with MRI-proven infarctions in the PTA territory. One patient had lesions in the unilateral thalamus and midbrain, the other two had lesions in the bilateral paramedian thalamus and unilateral midbrain, and the remaining patient had lesions in the unilateral thalamus and bilateral midbrain. Clinical manifestations depended on the variations of the size and extent of infarctions. Theanatomical variations of the PTA are discussed and suggested.

Two Cases of Primary Progressive Aphasia

Primary progressive aphasia (PPA) is an uncommon neurodegenerative syndrome characterized by a progressive deterioration of language, while nonverbal cognitive and other neurological functions of PPA are relatively preserved for a longer period. However, it still remains unclear whether PPA represents a distinct diagnostic entity or a precursor of global dementia syndrome. We report PPA cases that presented with a slowly progressive language dysfunction without disturbing other daily living activities for several years. Repeated neuropsychological tests revealed progres-sive deterioration of executive aspects of language and mild memory dysfunction, although their receptive language and nonverbal cognitive functions were relatively preserved. The imaging of the brain showed prominent atrophic changes in the left perisylvian and the adjacent temporal region. In considering the mild cognitive decline accompanied by language deterioration, we conclude that in these cases it is clinically heterogenous and may be parts of a spectrum of focal forms of non-Alzeimer type dementia.

A Case of Systemic Lupus Erythematosus Presented as Pseudotumor Cerebri Syndrome / 대한류마티스학회지

Pseudotumor cerebri syndrome is characterized clinically by raised intracranial pressure without ventriculomegaly. Several conditions known to interfere with CSF absorption pathways at the level of the arachnoid villi can produce the pseudotumor cerebri syndrome. Systemic lupus erythematosus (SLE) is an autoimmune, inflammatory and chronic disorder characterized by multi-organ involvement including the nervous system. Clinical evidence of central nervous system involvement includes headache, seizure, psychosis and altered mental status. However, pseudotumor cerebri syndrome has been reported infrequently as a primary feature of central nervous system involvement or a complication of SLE. A 19 year-old female was admitted with seizure preceded by headache and blurred vision. Ophthalmoscopic examination showed papilledema. The diagnosis of pseudotumor cerebri syndrome was confirmed by increased intracranial pressure(>550mmH2O) in the absence of any abnormal radiological findings of the brain. We described a 19-year-old girl whose first clinical manifestation was pseudotumor cerebri syndrome, which was diagnosed as SLE later. Therefore pseudotumor cerebri syndrome may be the part of the spectrum of clinical manifestation of SLE.

A Case of Systemic Lupus Erythematosus Presented as Pseudotumor Cerebri Syndrome / 대한류마티스학회지

Pseudotumor cerebri syndrome is characterized clinically by raised intracranial pressure without ventriculomegaly. Several conditions known to interfere with CSF absorption pathways at the level of the arachnoid villi can produce the pseudotumor cerebri syndrome. Systemic lupus erythematosus (SLE) is an autoimmune, inflammatory and chronic disorder characterized by multi-organ involvement including the nervous system. Clinical evidence of central nervous system involvement includes headache, seizure, psychosis and altered mental status. However, pseudotumor cerebri syndrome has been reported infrequently as a primary feature of central nervous system involvement or a complication of SLE. A 19 year-old female was admitted with seizure preceded by headache and blurred vision. Ophthalmoscopic examination showed papilledema. The diagnosis of pseudotumor cerebri syndrome was confirmed by increased intracranial pressure(>550mmH2O) in the absence of any abnormal radiological findings of the brain. We described a 19-year-old girl whose first clinical manifestation was pseudotumor cerebri syndrome, which was diagnosed as SLE later. Therefore pseudotumor cerebri syndrome may be the part of the spectrum of clinical manifestation of SLE.

A Case of Benign Intracranial Hypertension in Pseudopseudohypoparathyroidism

We describe a rare case of pseudopseudohypoparathyroidism (PPHP) with benign intracranial hypertension (BIH). A 16 year-old male presented with headache, vomiting, ocular pain, blurred vision, and diplopia following minor head trauma. He showed developmental delay, short stature and short metacarpals and phalanges, which suggested Albright's hereditary osteodystrophy. Neurologic examination revealed bilateral sixth cranial nerve palsy and bilateral papilledema. Lumbar puncure disclosed an elevated opening CSF pressure with normal biochemical and celluar find-ings. However, the levels of serum calcium, phosphorus and parathyroid hormone were within the normal limits and chromosomal analyses were normal. Brain MRI revealed normal ventricular size and no structural abnormality. We concluded that PPHP seemed to have a key role for the genesis of BIH in this case, although previous mild head trauma might have a precipitating effect.

A Case of Transient Cervical Dystonia following Cerebellar Hemorrhage

BACKGROUND: Cervical dystonia is a focal dystonia characterized by painful spasmodic contractions of the neck muscles, that result in an abnormal posture of the head. Usually the pathogenesis of the disorder is unknown. Secondary cervical dystonia caused by intracranial lesions, involving the basal ganglia, thalamus or brainstem, is very rare. This is the first report of a focal cervical dystonia following cerebellar hemorrhage in Korea. CASE: We describe a 77 year-old woman presented with vertigo and vomiting followed by mild ataxia in the right extremities and cervical dystonia. She had a 25-year history of right hemiparesis due to a stroke. On physical examination, her neck turned to the right and tilted to the left shoulder. Polygraphic study revealed continuous muscular contraction of the left sternocleidomastoid and trapezius muscles without EEG changes. A brain CT scan showed right cerebellar hemorrhage and low density lesions in the left putamen. CONCLUSION: We reported a rare case of cervical dystonia associated with cerebellar hemorrhage. Because she had an old left putaminal lesion, it is suggested that cervical dystonia seen in our patient may be caused by additional dysfunction of dentatorubrothalamocortical pathway to the pre-existing damage to the striatopallidothalamocortical pathway.

Outcome of Resective Surgery for Non-lesional Neocortical Temporal Lobe Epilepsy

BACKGROUND: Patients with neocortical temporal lobe epilepsy(NTLE) are reported to have less favorable outcome with anterior temporal lobectomy, and the prognostic factors for patients with non-lesional NTLE are not well defined. METHODS: We analyzed the multiple potential predictors of outcome for 26 consecutive medically intractable non-lesional NTLE patients who have had both extracranial and intracranial EEG monitoring during their presurgical evaluation prior to surgery: age, age at seizure onset, sex, seizure semiology, seizure duration and frequency, an etiology, the site of the surgery, the region of intracranial ictal onset, intracranial amobarbital procedure, neuropsychology test, neuroimaging, multiple subpial transection(MST) and surgical pathology. The average follow up period was 37.0+/-11.1(range 20-60) months. The outcome factors analyzed were compared to two types of outcome group; seizure free group(class I) and persistent seizure group(class II-IV) according to Engel's classification. RESULTS: Seventeen of 26 non-lesional NTLE patients(65.4%) were seizure free during follow up. Intracranial ictal onset confined to anterior temporal region only significantly predicted seizure free(p<0.001). Earlier seizure onset(p=0.08) and resection without MST(p=0.10) tended to be seizure free, but not statistically significant. CONCLUSION: Patients with non-lesional NTLE can be a good candidate for a tailored anterior temporal neocorticectomy with/without MST after intracranial EEG monitoring and functional mapping.

The Measurement of Bone Mineral Density in Parkinson's Disease

BACKGROUND: Osteoporosis, one of the most common metabolic bone disease, might be influenced by the severity of Parkinson's disease (PD). Objectives : We investigated the relationship between the Bone Mineral Density (BMD) and the severity of PD in postmenopausal and senile women. METHODS: We measured BMD of lumbar spine (L1-L4) by Dual energy X-ray absorptiometry (DEXA; Hologic QDR-4500A). We compared BMD between patient group (30 patients with PD) and control (183 postmenopausal and senile health women). The patients were divided into two groups according to osteoporosis and analyzed the following potential factors influencing BMD in PD; age, duration of symptom, age of onset, Hoehn and Yahr stage (H-Y stage), UPDRS motor score, duration of treatment, body mass index (BMI), dominant symptom such as tremor or rigidity. RESULTS: 1. BMD was significantly decreased with aging (p 0.05). CONCLUSION: Osteoporosis is related to H-Y stage, UPDRS motor score and BMI as well as aging in PD.

Paroxysmal kinesigenic choreoathetosis

Paroxysmal kinesigenic choreoathetosis (PKC) is characterized by short paroxysms of focal or generalized involuntary movement induced by sudden movements, and is a well-known disease in the neurologic literature, but only 4 cases have been reported in Korea. The purpose of the presentation is to clarify the clinical features of PKC in Korea. We clinically analyzed 20 patients with PKC between 1986 and 1994 at Yongdong Severance Hospital, Yonsei Medical Center, with a minimum of a 1 to 2 year follow-up period. There were 14 men and 6 women. The age at onset of the condition ranged from 8 to 17 years (mean, 13.1 years). Six patients (30%) had a family history of the condition and the mode of inheritance was suggestive of an autosomal recessive pattern. The involuntary movements seemed to be dystonic rather than choreoathetonic upon a mild attack, and the paroxysms were precipitated by sudden movements. The attacks occurred on one or both sides, and were often associated with dysarthria, upward gaze and sensory aura. Consciousness was never lost. Their duration were usually 10 to 30 seconds, and never more than two minutes. All laboratory tests including electroencephalographic and neuroimaging studies showed no abnormality. All patients responded well to diphenylhydantoin. PKC is not rare in Korea and has a benign course.

Cortical Atrophy Following Delayed Encephalopathy After Carbon Monoxide Poisoning

Twenty-three patients with delayed carbon monoxide (CO) encephalopathy completed a short-term follow-up brain computed tomographic (CT) study. Of them, 12 revealed low-density lesions in the cerebral white matter, 3 had pallidal low-density lesions, and one showed low-density lesions in the cerebral white matter and globus pallidus on initial CT scans. Of the 23 patients with follow-up brain CT scans, 9 (39.1%) showed more aggravating patterns with cortical atrophy . 8 with concomitant low-density lesions in the cerebral white matter. Age, sex, duration of unconsciousness during the anoxic episode and lucid interval did not appear to affect the presence of cortical atrophy, but the cerebral white matter low-density lesions on the initial CT scan seemed to be correlated with cortical atrophy after delayed CO encephalopathy. In conclusion, cortical atrophy after delayed CO sequelae is relatively common, and frequently associate with the cerebral white matter low-density lesions. Follow-up brain CT study will be needed to determine the development of cortical atrophy in all patients with delayed encephalopathy after CO poisoning.

A Case of Horizontal Canal Benign Paroxysmal Positional Vertigo

We report the clinical features in one patients with episodic positional vertigo and apogeotropic direction changing horizontal positional nystagmus that does not fatigue, beating to the right with the head turned to the left and beating to the left with the head turned to the right. This syndrome probably represents a horizontal semicircular canal variant of benign positional vertigo. Free-floating debris and Cupula attached debris in one horizontal semicircular canal may explain many of the clinical and oculographic findings.

The Longterm Prognostic Factors After Acute Cerebral Infartion

BACKGROUND & OBJECTIVES: There are few reports of affecting factors of the longterm prognosis after acute ischemic stroke. The aim of this prospective study was to determine which variables, of those easily measurable during the first 24 hours after stroke onset, would be predictors of longterm outcome. METHOD: Ninety-eight patients with an acute ischemic stroke were evaluated within 24 hours after symptom onset. The following potential affecting factors of outcome were prospectively collected : demographic datas, socioeconomic factors, risk factors of cerebrovascular accidents, clinical f indings as measured by the National Institute of Health (NIH) scale, laboratory findings and premorbid varibles rated according to modified Rankin score (mRS). We evaluated the outcome, assessed with mRS as good (mRS 3 or less) and poor (mRS 4, 5 or death) and follow up was undertaken at 6, 12, 18 and 24 months after onset. The statistical analysis was performed by means of SPSS/PC+package to determine the longterm prognostic factors. RESULTS: The multiple stepwise regression analysis revealed that the outcome depend on NIH scale(p<0.001), age (p<0.01), previous stroke(p<0.01), loss of consciousness (LOC) at onset (p

Two Cases of Stimulus Sensitive Spinal Myoclonus

Myoclouns is a complicated and poorly-understood phenomenon caused by many pathological conditions. Myoclonus which is triggered by sensory stimuli has been termed reflx myoclonus and divided into cortical, reticular and spinal types depending on the generator sites. Segmental myoclonus of spinal origin consists of synchronous rhythmical jerks confined to a group of muscles supplied by either one segment or several contiguous segments of the spinal cords. We present two cases of spinal reflex myoclonus. One was 50 years old female who showed complex myoclonic jerks involving the abdominal wall and paraspinal muscibs resembling 'belly dance'. The other was 26 years old male who showed intermittent synchronous upper cervical axial muscle contractions. In both cases, jerks were increased with certain stimuli, but disappeared during sleep. They showed normal EEGs and absence of enhancement of SSEPS.

A Case of Recurrent Localized Myositis

We experienced a 41-year-old male patient with the recurrent painful nodular swelling of muscles in both lower limbs for 4 years. The mass did not accompanied other general symptoms of muscle weakness and spontaneously regressed. On pathologic finding, there is marked inflammatory cell infiltration with muscle fiber destruction and regeneration. Analyzing the literature, we proposed the diagnosis of recurrent localized myositis.

A Case of Suspected Post-Irradiation Delayed Necrotizing Leukoencephalopathy

Therapeutic irradiation may produce a variety of adverse side effects on the central and the peripheral nervous systems. These adverse effects are divided into two main groups; transient and permanent. Four major clinical syndromes have been described in relation to permanent radiation injury of the nervous system: encephalopathy, cranial neuropathy, myelopathy, and peripheral neuropathy. Among them, delayed encephalopathy represents the major hazard in CNS irradiation. The characteristic MR imaging pattern of radiation induced delayed necrotizing leukoencephalopathy is symmetric, high-signal foci on T2WI in the periventricular white matter. We report a case of suspected post-irradiation leukoencephalopathy showed progressive mental change, quadriparesis, and urinary incontinence with characteristic MR findings who had taken therapeutic irradiation 12 months ago.

Intravenous Immunoglobulin Therapy in Guillain-Barre Syndrome

High dose intravenous immunoglobulin (IVIg) therapy can improve the clinical course of several immune mediciated diseases. We evaluated clinical effects and side effects of IVIg in Guillain-Barre syndrome (GBS). 19 Patients with GBS were studied prospectively in a placebo-controlled trial. 11 Patients were received high dose IVIg (400mg/kg for 5 days) and controls received only conservative treatment. The disability scores using modified Rankin scores before and after treatment of each group were compared. Four weaks later, mean Rankin Score of IVIg group was 2.5 + 0.7 and control group was 3.3+ 0.5which showed significant difference(p<0.05). There were no serious advers effer of promote early improvement with safety in acute phase of Guillan-Barre syndrome.