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Neurocutaneous Melanosis Presenting as Chronic Partial Epilepsy.

Byoung Seok YE ; Yang Je CHO ; Sang Hyun JANG ; Byung In LEE ; Kyoung HEO ; Hyun Ho JUNG ; Jin Woo CHANG ; Se Hoon KIM

Journal of Clinical Neurology.2008;4(3):134-137. doi:10.3988/jcn.2008.4.3.134

BACKGROUND: Neurocutaneous melanosis (NCM) is a rare neurocutaneous syndrome characterized by the presence of multiple congenital melanocytic nevi (CMN) and the proliferation of melanocytes in the central nervous system, usually involving the leptomeninges. Chronic partial epilepsy as a sole manifestation is rare in NCM. CASE REPORT: A 32-year-old man suffering from chronic partial epilepsy presented with multiple CMN on his trunk and scalp. Brain MRI demonstrated a focal lesion in the right amygdala that was consistent with interictal epileptiform discharges in the right temporal region on electroencephalography (EEG). An anterior temporal lobectomy was performed, and the pathology investigation revealed numerous melanophages in the amygdala. The patient was seizure-free after surgery. CONCLUSIONS: We report a patient with NCM presenting as chronic partial epilepsy who was successfully treated by anterior temporal lobectomy.
Adult ; Amygdala ; Anterior Temporal Lobectomy ; Brain ; Central Nervous System ; Electroencephalography ; Epilepsies, Partial ; Humans ; Melanocytes ; Melanosis ; Neurocutaneous Syndromes ; Nevus, Pigmented ; Scalp ; Stress, Psychological

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Warfarin-Induced Intracerebral Hemorrhage Associated with Microbleeds.

Gha Hyun LEE ; Sun U KWON ; Dong Wha KANG

Journal of Clinical Neurology.2008;4(3):131-133. doi:10.3988/jcn.2008.4.3.131

BACKGROUND: Microbleeds (MBs), proposed as a biomarker for microangiopathy, have been suggested as a predictor of spontaneous or thrombolysis-related intracerebral hemorrhage (ICH) in acute ischemic stroke. However, the relationship between MBs and warfarin-induced ICH is not clear. CASE REPORT: We describe two patients who developed warfarin-induced ICH at the site of MBs documented in previous MRI. CONCLUSIONS: The presence of MBs might increase the risk of ICH after warfarin use in ischemic stroke patients. A large cohort study is required to confirm the relationship of MBs with warfarin-induced ICH.
Cerebral Hemorrhage ; Cohort Studies ; Humans ; Magnetic Resonance Imaging ; Stroke ; Warfarin

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A Double Mutation of the Ryanodine Receptor Type 1 Gene in a Malignant Hyperthermia Family with Multiminicore Myopathy.

Seul Ki JEONG ; Dong Chan KIM ; Yong Gon CHO ; Il Nam SUNWO ; Dal Sik KIM

Journal of Clinical Neurology.2008;4(3):123-130. doi:10.3988/jcn.2008.4.3.123

BACKGROUND AND PURPOSE: At least 100 Ryanodine receptor type 1 (RYR1) mutations associated with malignant hyperthermia (MH) and central core disease (CCD) have been identified, but 2 RYR1 mutations accompanying multiminicore myopathy in an MH and/or CCD family have been reported only rarely. METHODS: Fifty-three members of a large MH family were investigated with clinical, histopathologic, RYR1 mutation, and haplotyping studies. Blood creatine kinase (CK) and myoglobin levels were also measured where possible. RESULTS: Sequencing of the entire RYR1 coding region identified a double RYR1 mutation (R2435H and A4295V) in MH/CCD regions 2 and 3. Haplotyping analysis revealed that the two missense heterozygous mutations (c.7304G>A and c.12891C>T) were always present on a common haplotype allele, and were closely cosegregated with histological multiminicores and elevated serum CK. All the subjects with the double mutation showed elevated serum CK and myoglobin, and the obtained muscle biopsy samples showed multiminicore lesions, but only two family members presented a late-onset, slowly progressive myopathy. CONCLUSIONS: We found multiminicore myopathy with clinical and histological variability in a large MH family with an unusual double RYR1 mutation, including a typical CCD-causing known mutant. These results suggest that multiminicore lesions are associated with the presence of more than two mutations in the RYR1 gene.
Alleles ; Biopsy ; Clinical Coding ; Creatine Kinase ; Haplotypes ; Humans ; Malignant Hyperthermia ; Muscles ; Muscular Diseases ; Myoglobin ; Myopathies, Structural, Congenital ; Myopathy, Central Core ; Ophthalmoplegia ; Ryanodine ; Ryanodine Receptor Calcium Release Channel

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Quality of Life after Epilepsy Surgery in Korea.

Smi CHOI-KWON ; Chun Kee CHUNG ; Sang Kun LEE ; Jimi CHOI ; Kihye HAN ; Eun Hyun LEE

Journal of Clinical Neurology.2008;4(3):116-122. doi:10.3988/jcn.2008.4.3.116

BACKGROUND AND PURPOSE: Temporal changes in the quality of life (QOL) and the underlying factors after epilepsy surgery might be specific to Korea, where social stigma toward patients with epilepsy is still pronounced. METHODS: The seizure characteristics, number of antiepileptic drugs (AEDs), and the presence of stigma, anxiety, and depression were assessed before and after surgery (at 6 months and around 2 years) in 32 surgery patients and 32 nonsurgery patients. The QOL was compared between these groups using the Epilepsy Surgery Inventory-55 questionnaire. The factors affecting QOL were also evaluated. RESULTS: The scores in the mental, physical, and role-functioning domains were significantly higher at 6 months (all p<0.01) and around 2 years (all p<0.01) than at baseline in the surgery group but not in the nonsurgery group. The factors related to QOL differed at the two follow-up times, with seizure freedom being important at 6 months, and AEDs and depression being important at around 2 years. CONCLUSIONS: A marked increase in QOL in our population was observed after epilepsy surgery. Although the small sample limits the interpretation of the results, the QOL change in our surgery patients shows similar trends to those reported in Western countries. A full understanding of underlying factors related to QOL might aid the development of optimal strategies for improving the long-term postsurgery QOL in this population.
Anticonvulsants ; Anxiety ; Depression ; Epilepsy ; Follow-Up Studies ; Freedom ; Humans ; Korea ; Quality of Life ; Seizures ; Social Stigma ; Surveys and Questionnaires

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Association of Initial Infarct Extent and Progressive Motor Deficits in Striatocapsular Infarction.

Heui Soo MOON ; Yong Bum KIM ; Bum Chun SUH ; Yu Sam WON ; Kwang Yeol PARK ; Pil Wook CHUNG

Journal of Clinical Neurology.2008;4(3):111-115. doi:10.3988/jcn.2008.4.3.111

BACKGROUND AND PURPOSE: Progression of motor deficits in the acute period is frequently observed in patients with subcortical striatocapsular infarctions. Therefore, we sought to determine the factors associated with early motor progression in patients with infarcts confined to the striatocapsular region. METHODS: We studied 80 consecutive patients with striatocapsular-region infarction, as defined by clinical and MRI criteria, within 24 hours after stroke onset. Motor progression was defined as an increase of >2 points in the motor items of the National Institutes of Health Stroke Scale (NIHSS) within 7 days of stroke onset. The study population was divided into patients with and without motor progression, and risk factors, clinical features, and brain MRI/magnetic resonance angiograpy (MRA) findings were compared between these groups. RESULTS: Motor progression was observed in 40% of the 80 patients. The independent variables associated with motor progression were a history of hypertension (OR=7.8, 95% CI=1.5-39.8, p=0.013) and an initial infarct extent of > or =15 mm (OR=9.2, 95% CI=1.8-45.7, p=0.006). However, there were no differences in the initial NIHSS score, other stroke risk factors, vascular stenosis pattern, or cardioembolic source. CONCLUSIONS: Early motor progression in patients with striatocapsular-region infarction is associated with the initial extent of the lesion. However, the stroke mechanism and vascular stenosis did not differ between the motor progression and stable groups.
Brain ; Constriction, Pathologic ; Humans ; Hypertension ; Infarction ; Magnetic Resonance Imaging ; National Institutes of Health (U.S.) ; Risk Factors ; Stroke

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Residual Dizziness after Successful Repositioning Treatment in Patients with Benign Paroxysmal Positional Vertigo.

Jung Im SEOK ; Hyo Min LEE ; Ji Hoon YOO ; Dong Kuck LEE

Journal of Clinical Neurology.2008;4(3):107-110. doi:10.3988/jcn.2008.4.3.107

BACKGROUND AND PURPOSE: Benign paroxysmal positional vertigo (BPPV) is the most common form of vertigo. Although the repositioning maneuver dramatically improves the vertigo, some patients complain of residual dizziness. We evaluated the incidence and characteristics of persistent dizziness after successful particle repositioning and the clinical factors associated with the residual dizziness. METHODS: We performed a prospective investigation in 49 consecutive patients with confirmed BPPV. The patients were treated with a repositioning maneuver appropriate for the type of BPPV. Success was defined by the resolution of nystagmus and positional vertigo. All patients were followed up until complete resolution of all dizziness, for a maximum of 3 months. We collected data on the characteristics and duration of any residual dizziness and analyzed the clinical factors associated with the residual dizziness. RESULTS: Of the 49 patients, 11 were men and 38 were women aged 60.4+/-13.0 years (mean +/-SD), and 30 (61%) of them complained of residual dizziness after successful repositioning treatment. There were two types of residual dizziness: continuous lightheadedness and short-lasting unsteadiness occurring during head movement, standing, or walking. The dizziness lasted for 16.4+/-17.6 days (range=2-80 days, median=10 days). A longer duration of BPPV before treatment was significantly associated with residual dizziness (p=0.04). CONCLUSIONS: Residual dizziness after successful repositioning was observed in two-thirds of the patients with BPPV and disappeared within 3 months without specific treatment in all cases. The results indicate that early successful repositioning can reduce the incidence of residual dizziness.
Aged ; Dizziness ; Female ; Head Movements ; Humans ; Incidence ; Male ; Prospective Studies ; Vertigo ; Walking

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Cognitive Effects of Antiepileptic Drugs.

Sung Pa PARK ; Soon Hak KWON

Journal of Clinical Neurology.2008;4(3):99-106. doi:10.3988/jcn.2008.4.3.99

Antiepileptic drugs (AEDs) can adversely affect cognitive function by suppressing neuronal excitability or enhancing inhibitory neurotransmission. The main cognitive effects of AEDs are impaired attention, vigilance, and psychomotor speed, but secondary effects can manifest on other cognitive functions. Although the long-term use of AEDs can obviously elicit cognitive dysfunction in epilepsy patients, their cognitive effects over short periods of up to a year are inconclusive due to methodological problems. In general, the effects on cognition are worse for older AEDs (e.g., phenobarbital) than for placebo, nondrug condition, and newer AEDs. However, topiramate is the newer AED that has the greatest risk cognitive impairment irrespective of the comparator group. Since the cognitive impact of AEDs can be serious, clinicians should be alert to adverse events by evaluating cognitive function using screening tests. Adverse cognitive events of AEDs can be avoided by slow titration to the lowest effective dosage and by avoiding polytherapy.
Anticonvulsants ; Cognition ; Epilepsy ; Fructose ; Humans ; Mass Screening ; Neurons ; Synaptic Transmission

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Blood Pressure in Acute Ischemic Stroke.

Michael MCMANUS ; David S LIEBESKIND

Journal of Clinical Neurology.2016;12(2):137-146. doi:10.3988/jcn.2016.12.2.137

Hypertension is present in up to 84% of patients presenting with acute stroke, and a smaller proportion of patients have blood pressures that are below typical values in the context of cerebral ischemia. Outcomes are generally worse in those who present with either low or severely elevated blood pressure. Several studies have provided valuable information about malignant trends in blood pressure during the transition from the acute to the subacute phase of stroke. It is not uncommon for practitioners in clinical practice to identify what appear to be pressure-dependent neurologic deficits. Despite physiologic and clinical data suggesting the importance of blood pressure modulation to support cerebral blood flow to ischemic tissue, randomized controlled trials have not yielded robust evidence for this in acute ischemic stroke. We highlight previous studies involving acute-stroke patients that have defined trends in blood pressure and that have evaluated the safety and efficacy of blood-pressure modulation in acute ischemic stroke. This overview reports the current status of this topic from the perspective of a stroke neurologist and provides a framework for future research.
Blood Pressure* ; Brain Ischemia ; Humans ; Hypertension ; Hypotension ; Neurologic Manifestations ; Stroke*

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Considerations When Subtyping Ischemic Stroke in Asian Patients.

Oh Young BANG

Journal of Clinical Neurology.2016;12(2):129-136. doi:10.3988/jcn.2016.12.2.129

Both the incidence and prevalence of stroke in Asia are steadily increasing, and the burden of stroke is particularly high in Asian countries. Although strokes in Asians and Caucasians share many common features, there are some differences that are probably due to differences in lifestyle and genetic background. While there have been advances in the stroke classification system, the assignment of Asian stroke patients to etiological categories has received little attention. The current classification system may not be well suited to Asian patients with ischemic stroke because the proportions and relative importance of stroke subtypes may differ with race and ethnicity. This review addresses concerns about the use of the current stroke classification system in Asian patients with ischemic stroke, and proposes a classification system that is more specific to the Asian population, in conjunction with discussing advances in diagnostic techniques.
Asia ; Asian Continental Ancestry Group* ; Classification ; Continental Population Groups ; Humans ; Incidence ; Life Style ; Prevalence ; Stroke*

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Erratum to: Severe Paraneoplastic Parkinsonism: A Rare Cause Revealing Breast Cancer.

Nabil ABIDA ; Houyam TIBAR ; Adil BOUDHAS ; Jawad OUMERZOUK ; Ahmed BOURAZZA

Journal of Clinical Neurology.2017;13(4):448-448. doi:10.3988/jcn.2017.13.4.448

At the request of the authors, the names of the authors should be as follow: Abida N, Tibar H, Boudhas A, Oumerzouk J, Bourazza A.

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