Identification of differentially expressed cDNAs in Acanthamoeba culbertsoni after mouse brain passage

Free-living amoebae of the genus Acanthamoeba are causative agents of granulomatous amebic encephalitis and amebic keratitis. Because the virulence of Acanthamoeba culbertsoni cultured in the laboratory is restored by consecutive brain passages, we examined the genes induced in mouse brain-passaged A. culbertsoni by differential display reverse transcriptase polymerase chain reaction (DDRT-PCR). Enhanced A. culbertsoni virulence was observed during the second mouse brain passage, i.e., infected mouse mortality increased from 5% to 70%. Ten cDNAs induced during mouse brain passage were identified by DDRT-PCR and this was confirmed by northern blot analysis. BlastX searches of these cDNAs indicated the upregulations of genes encoding predictive NADH-dehydrogenase, proteasomal ATPase, and GDP-mannose pyrophosphorylase B, which have previously been reported to be associated with A. culbertsoni virulence factors.

Neuromyeloencephalopathy Induced by Inadvertent Intrathecal Vincristine Injection

Accidental intrathecal vincristine instillation is extremely toxic to the central nervous system. If given intrathecally, it produces a rapidly ascending, usually fatal, neuromyeloencephalopathy. This is a case where a 10-year-old girl received an inadvertent intrathecal administration of vincristine. The clinical course was rapidly progressive and resulted in comatose and vegetative state. Magnetic resornance image at post-injection 11 day showed high signal intensity lesion in cerebellar hemisphere and vermis adjacent to CSF passage third and fourth ventricle on T2 weighted images.

The Somatosensory Evoked Potentials and Nerve Conduction Studies in Patients with Diabetic Polyneuropathy

BACKGROUND: The nerve conduction study (NCS) is one of the most important diagnostic tools used to evaluate the function of large myelinated nerve fibers. However, the NCS in patients with diabetic polyneuropathy, who have senso-ry and motor abnormalities on neurological examination, are frequently found to be within normal limits. Somatosensory Evoked Potentials (SEP), F-waves, and NCS were performed to determine their usefulness in detecting electrophysiologic abnormalities in the early stages of patients with diabetic polyneuropathy. METHODS: Posterior tibial SEP (PTSEP) studies were performed in thirty patients with clinically suspected diabetic polyneuropathy who had with-in normal values of NCS involving the upper and lower extremities. PTSEP, F-wave, and NCS were also performed in age and height matched eighteen normal participants. RESULTS: The mean latencies of spinal evoked potentials (T12) and cortical evoked potentials (P1) were significantly prolonged in the diabetic patients compared to the control group (p 0.05). CONCLUSIONS: We found the SEP study to be a useful diagnostic test for detecting diabetic polyneuropathy.

Mean Platelet Component to Measure Platelet Activation in Ischemic Stroke: Preliminary Study

BACKGROUND: Abnormal platelet activation has been identified in several disorders characterizedby vascular patholo-gy including coronary artery disease, Alzheimer disease, myeloproliferative disorders, diabetes, preeclampsia, inflam-matory bowel disease and glomerular disease. Antiplatelet therapy has been valuable in the management of some of these conditions. The aim of this study is to verify usefulness of mean platelet component (MPC) concentration as a marker of thrombotic process in patients with cerebral infarction. Our hypothesis is that MPC as measured by the ADVIA(R) 120 hematology system is used to detect and monitor platelet activation associated with thrombotic process of ischemic stroke. METHODS: To study the existence of platelet activation at the onset of cerebral infarction, mean platelet concentration of platelets were measured daily during post-stroke 10 days. Thirty-four acute thrombotic cerebral infarction and seventeen age-matched healthy persons were selected for this study. To investigate the time course of the platelet MPC changes observed in stroke patients, the blood samplings for MPC measuring were done and analyzed on the ADVIA 120(R) system. RESULTS: There was a statistically significant decrease in MPC concentration of the platelets at post-stroke 3rd to 7th day compared to the control group ( p < 0.05). CONCLUSIONS: We conclude that a reduction of MPC as measured by the ADVIA 120(R) hematology system may be used to detect and monitor thrombotic process associated with platelet activation in ischemic stroke.

Temporal Variant of Frontotemporal Dementia: A Case of Semantic Dementia

Semantic dementia is a rare, distinct form of frontotemporal lobar degeneration, characterized by a deficit in semantic memory with relative preservation of attention and executive functions. We report a case of semantic dementia that pre-sented with a 3-year history of progressive word-finding difficulty and prosopagnosia. Brain MRI showed prominent atrophic changes in the left temporal region and neuropsychological tests demonstrated semantic memory deficits.

Cerebrospinal Fluid Aspartate Aminotransferase in Alzheimer Disease and Vascular Dementia

BACKGROUND: To evaluate usefulness of cerebrospinal aspartate aminotransferase(AST) as a biologic marker for differentiation of Alzheimer's disease(AD) and Vascular dementia(VD) METHODS: A consecutive series of patients who met either the criteria of the National Institute of Neurological Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association(NINCDSADRDA) for probable AD or National Institute of Neurological Disorders and Stroke and the Association Internationale pour la Recherche et l'Enseignement en Neurosciences(NINDS-AIREN) criteria for porbable VD were included in the study. Enzymatic determinations in cere brospinal fluid of aspartate aminotransferase in cerebrospinal fluid of aspartate aminotransferase and serologic analysis of apolipoprotein E were performed in 17 patients with AD and in 15 patients with VD. And we compared CSF AST of AD with that of VD. RESULTS: We found no difference of CSF AST concentration between patients with Alzheimer's disease and vascular dementia. Cerebrospinal AST activity also did not correlate with K-MMSE score, serum AST activity, Functional inde-pence measure(FIM) as a ADL(Activity of daily living), and presence of Apolipoprotein E4 allele in AD. Only serum AST of VD shows correlation with CSF AST. CONCLUSION: These findings suggest that cerebrospinal AST concentration is not useful maker for differentiation between AD and VD.

Neuropsychological Differentiation between Alzheimer's Disease and Vascular Dementia

BACKGROUND: Neuropsychological Differentiation between Alzheimer's disease and vascular dementia has been controversial. The aim of this study was to detect neuropsychological differences between Alzheimer's disease (AD) and vascular dementia (VD). METHODS: Twenty one patients who met the criteria for probable AD according to the National Institute of Neurological Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association (NINCDS-ADRDA) and 22 patients who met criteria for probable VD according to the National Institute of Neurological Disorders and Stroke and the Association Internationale pour la Recherche et l'Enseignement en Neurosciences (NINDS-AIREN), were included in this study. These two dementia groups were matched for age, sex, education, Mini-Mental State Examination score, and severities of dementia. All patients underwent the Seoul Neuropsychological Screening Battery (SNSB). RESULTS: Patients with VD showed greater deficits in verbal fluency (P<.05) than AD patients. However, AD patients, compared with VD patients, showed greater deficits in verbal memory functions including the 3 words recall task. Otherwise, no significant between-group differences were found in task results. CONCLUSIONS: Neuropsychologically, patients with VD differ from patients with AD only in a few cognitive domains. (J Korean Neurol Assoc 19(2):143~148, 2001)

Neuropsychological Differentiation between Alzheimer's Disease and Vascular Dementia

BACKGROUND: Neuropsychological Differentiation between Alzheimer's disease and vascular dementia has been controversial. The aim of this study was to detect neuropsychological differences between Alzheimer's disease (AD) and vascular dementia (VD). METHODS: Twenty one patients who met the criteria for probable AD according to the National Institute of Neurological Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association (NINCDS-ADRDA) and 22 patients who met criteria for probable VD according to the National Institute of Neurological Disorders and Stroke and the Association Internationale pour la Recherche et l'Enseignement en Neurosciences (NINDS-AIREN), were included in this study. These two dementia groups were matched for age, sex, education, Mini-Mental State Examination score, and severities of dementia. All patients underwent the Seoul Neuropsychological Screening Battery (SNSB). RESULTS: Patients with VD showed greater deficits in verbal fluency (P<.05) than AD patients. However, AD patients, compared with VD patients, showed greater deficits in verbal memory functions including the 3 words recall task. Otherwise, no significant between-group differences were found in task results. CONCLUSIONS: Neuropsychologically, patients with VD differ from patients with AD only in a few cognitive domains. (J Korean Neurol Assoc 19(2):143~148, 2001)

A Reversible Posterior Leukoencephalopathy Syndrome

Reversible posterior leukonecephalopathy syndrome RPLS), a neurologie disorder associated with evidence of posterior cerebral edema on neuroimaging studies, has been described in both adults and children. Conditions predisposing to RPLS include malignant hypertension, renal dysfunction, toxemia of pregnancy, interferon therapy, and the use of immunosuppressive agents. When associated with acute hypertension, RPLS typically occurs concurrently with the fulminant clinical syndrome of hypertensive encephalopathy We describe occipital lobe seizures, in the setting of only moderate elevations of blood pressure, as the major clinical manifestation of RPLS.

Usefulness of Quantified-EEG in Alzheimer's Disease

BACKGROUND: The conventional electroencephalography (EEG) is commonly used as an aid in the diagnosis of Alzhiemer's disease (AD). Recently developed quantitative electroencephalography (qEEG) provides data that are not achievable by conventional EEG. Nevertheless, the clinical reliability and usefulness of spectral analysis and topographic mapping is still a matter of controversy in the diagnosis of Alzheimer's disease. The aim of this study was to compare the topographical quantitative EEG (qEEG) changes between elderly controls and AD patients. METHODS: We analyzed the absolute, relative spectra power and occipital peak frequency taken from 16 derivations by averaging twenty-2-sec epoch in elderly controls and AD patients. After logarithmic transformations of absolute and relative power, a statistical test was done and occipital peak frequency was compared with each other. RESULTS: (1) Compared with normal controls, AD patients had a significantly lower alpha and beta spectra power as well as a significant higher delta spectra power. (2) In AD patients, the peak frequency in occipital lead had a significantly lower frequency than that of elderly controls. (3) Korean Mini-Mental State Examination(K-MMSE) scores were closely related in delta and alpha band power in nearly all electric leads. (4) Compared to absolute power, relative power was a more sensitive tools in detecting changes of EEG in AD patients. CONCLUSIONS: This study suggests that qEEG is a useful tool for detecting the electroencephalographic changes of AD, and correlated with the severity of cognitive dysfunction. Moreover, absolute and relative spectra power is complementary in the clinical utility of qEEG.

Clinical Characteristics of Geriatric Patients Admitted to Yongin Hyoja Geriatric Hospital

BACKGROUND: As the size of the elderly population has increased compared to the hole, there has been growing concern over potential health problems and geriatric hospitals for elderly people. However, despite the emerging needs for geriatric hospitals, there is no data on the clinical characteristics of patients being admitted to geriatric hospitals. To analyze the characteristics of elderly patients admitted to a geriatric hospital, we studied the medical records of patients admitted to Yong-in Hyoja Geriatric Hospital during a recent 20 months period. By clarifying the clinical characteristics of elderly patients admitted to a geriatric hospital, the basic data for understanding and treating elderly patients could be made. METHODS: We analyzed the medical records of 345 patients who were admitted to Yong-in Hyoja Geriatric Hospital from November 1997 to July 1999. The diagnosis of patients was largely subgrouped according to the existence of dementia, and subsequent detailed diagnoses were made. RESULTS: (1) The average age of patients was 74.59 years and there were slightly more females than males admitted (ratio, 1.15:1). (2) Most of the patients suffered from dementia (62.1%), of which vascular dementia was the most common(31.6%), followed by Alzheimer dementia(19.15%). (3) Compared to vascular dementia, patients with Alzheimer dementia had more severe cognitive dysfunction, psychosis, and agitation, while patients with vascular dementia had more problems in activities of daily living (ADL) and were more depressed than Alzheimer dementia (4) The average hospitalization duration was 3.54 months and incidence of significant illness after admission was 29.8%. The mortality rate was 10.9%. (5) The most common cause of death was infection (45.2%), followed by cardiovascular problems(16.2%). CONCLUSIONS: In addition to significant illnesses and mortality after admission, most patients admitted to geriatric hospitals have severe cognitive and behavioral problems. Therefore, adequate medical and neurological assessment and treatment for the aged is necessary.

Beck Depression Inventory in Temporal Lobe Epilepsy with Hippocampal Atrophy: Relation to Lesion Laterality

BACKGROUND: Depression is found more frequently in epileptic patients and tends to be more severe in those with temporal lobe epilepsy, than in patients with comparable chronic neurologic diseases or physical handicaps. The purposes of this study were to evaluate (1) the characteristics and frequency of depression in group of temporal lobe epileptic out-patients with hippocampal atrophy; (2) the relationship between depression and the laterality of hippocampal atrophy, and (3) the possible correlation between depression and the duration of epilepsy, sex, education, age, occupation, seizure frequency, and other seizure variables. METHODS: We included 40 temporal lobe epilepsy patients with unilateral hippocampal atrophy on MRI study. We used the Beck Depression Inventory to measure the level of depression. The results were compared with those of 50 normal controls. RESULTS: (1) Epilepsy patients with hippocampal atrophy indicated more severe depression than the normal controls. If we consider the cut-off score for depression as being more than 21 points of the Beck Depression Inventory score, then the frequency of depression in TLE with hippocampal atrophy would be 45% compared to the 14% in controls. (2) Occupation and seizure frequencies were factors related to severe depression. However, the age, age of onset, duration of illness, religion, education, and multi-drug therapy, were not related to the severity of depression. (3) Scores on the BDI questions representing mood symptoms were significantly higher in the left TLE group. However, the frequencies of those representing vegetative and somatic symptoms were not different between the two groups. Self-reproach symptoms increased equally in both temporal lobe epilepsy groups. (4) There were no clear associations between depressive disorders and the laterality of epileptic lesions in the TLE patients. CONCLUSIONS: We found no clear association between the affective disorders in epilepsy and the site of epileptic lesions.

Add-On Therapy of Lamotrigine in Refractory Partial Epileptic Patients Taking Carbamazepine

BACKGROUND: Lamotrigne (LTG) is a newly developed antiepileptic drug which has shown to'be effective for medically intractable partial seizures. LTG was recently introduced to Korea but its clinical efficacy has not been investigated yet. METHODS: We assigned 34 medically intractable localization related epileptic patients taking maximally tolerable dose of carbarmazepine(CBZ). The study protocol consisted of 12 weeks of baseline phase, 4 weeks of phase I (drug -adjustment phase) and 8 weeks o f phase II (maintenance of LTG 200mg/day) After phase II, eligible patients entered into long-term therapy. Two patients dropped out during phase II for adverse event (AE) in one, and AE and poor seizure control in the other. RESULTS: Intention to treat analysis of the seizure outcome after 12 weeks of LTG add-on therapy showed mean seizure frequency reduction of 23.6% (p=O.006). More than 50% seizure frequency reduction was seen in 9 of 34 patients (26.4%), which was comparable to the results of previous clinical trials. On the other hand, the incidence of AE were quite high, which was developed in 27 patients. Dizziness with or without blurred vision and/or diplopia were the most common AE and occurred in 64.7%, which improved promptly by either reduction of CBZ or LTG doses. Twenty-two patients entered into long-term therapy and 18 patients showed either maintenance or more. LTG add-on therapy did not show any significant alterations of baseline Lab. Tests. DISCUSSION: LTG was an effective and safe new antiepileptic drug. However, about two-third of our patients developed A. E. similar to CBZtoxicity, which should be carefully considerd for treating patients taking maximally tolerable CBZ therapy. The proportion of patients taking LTG 300mg/day or more was very low in this study, which suggested the racial difference of tolerability to LTG.

Dystonia Following Head Trauma 1 Case

A 14-year-old male developed generalized dystonia following head trauma caused by a traffic accident. The tremulous movements with abnormal posture started in the left extremities at about 45 days, then spreaded to the right side at 4 months, and eventually to the neck turing to the right side at 13 months after the accident. He also showed mild degree of intellectual impairment, but no other focal neurological signs and symptoms. T2-weighted brain MRI revealed multiple high signal lesions scattered over corpus callosum, deep white matter and right thalamus.

A Clinical Study of Metastatic Spinal Epidural Tumor

The authors analysed 40 cases of metastatic spinal epidural tumor who admitted to the Wonju Christian hospital and Severance hospital, Yonsei University from January, 1989 to September, 1993. The results were summarized as follows: 1. The mean age was 54.7 years old and the metastatic spinal epidural tumor was most common in the 7th decade of age (35.0%). 2. The most common primary tumor was lung cancer (37.5%), and the rests were hepatoma (15.0%), leukemia (7.5%), stomach cancer (7.5%), lymphoma (5.0%) and pancreas cancer (5.0%), while 4 cases (10.0%) were of unknown primary tumor. 3. The level of the involved spine was thoracic (67.5%), lumbosacral (25.0%), and cervical (7.5%) in orders. 4. The most common initial symptom was pain and the common symptoms at diagnosis were pain, weakness, sensory loss and voiding difficulty. 5. In radiologic studies, plain X-ray showed bone involvement in 70.6%, 74.1% in bone scan and 87.5% in MRI respectively. 6. Signal intensity of tumor in MRI was iso or low on T1WI and high on T2WI. 7. Response to treatment was excellent in 60%, especially in early treated group within 5 days.

Acute Pandysautonomia with Somatic Nerve Involvement

Acute panautonomic neuropathy characterized by selective involvement of sympathetic and parasympathetic nerves with complete or relative sparing of somatic motor and sensory functions was first described by Young et al in 1969. We experienced acute pandysautonomia with peripheral neuropathy. The serial electrophysiologic studies revealed progressive periphear axonal polyneuropathy. Autonomic function test suggested dysautonomia of selective postganglionic lesion in 1 case and pandysautonomia in 3 cases. Our four cases wree Acute panautonomic neuropathies which simultaneously developed sensorimotor polyneuropathy. All Laps were normal except elevated CSF protein without pleocytosis. Recovery was poor in general, but 34-years-old female patient was markedly improved after plasmapheresis. Therefore we recommend immunologic treatment such as plasmapheresis or pulse therapy in early stage of progressive pandysautonomia.

Moyamoya Disease Presenting with Chorea

Two patients with moyamoya disease are reported whose initial and predominant manifestations were choreic movements. Choreic movements were recurrent and were often tnggered by excitement, emotional tension, or insomnia There were occurred unilaterally Moyamoya disease must be considered in the differential diagnosis of acquired chorea in young adults.

A Case of Perioptic Neuritis associated with Cryptococcal Infection A

Perioptic Neuritis ls a very rare clinical condition, in which optic disc swelling and inflammation is seen despite of normal intracranial pressure and visual acuity. These condition can be seen in syphilis, sarcoidosis, viral encephalitis, fungal infection. We have seen that the 45 years old woman with perioptic neuritis shows papilledema without IICP in spinal tapping, thought to be due to cryptococcal meningitis.