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Isolated Weakness of Middle, Ring, and Little Fingers due to a Small Cortical Infarction in the Medial Precentral Gyrus.

Young Su HAN ; Sang Won HA ; Jeong Seon CHO ; Sang Eun PARK ; Jung Mee KIM ; Jeong Ho HAN ; Eun Kyoung CHO ; Doo Eung KIM

Journal of Clinical Neurology.2006;2(2):146-148. doi:10.3988/jcn.2006.2.2.146

Small cortical strokes can produce predominant isolated weakness in a particular group of fingers: radial or ulnar. The traditional views are of point-to-point representations of each finger to neurons located in the precentral gyrus of the motor cortex such that the neurons of the radial fingers are located laterally and those of the ulnar fingers are located medially. We present a case of isolated weakness of middle, ring, and little fingers due to a small cortical infarction in the medial precentral gyrus.
Fingers* ; Infarction* ; Motor Cortex ; Neurons ; Stroke

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Diffusion-Weighted MRI in Recurrent Wernicke's Encephalopathy: a Remarkable Cerebellar Lesion.

Jung Eun KIM ; Tae Hyung KIM ; In Kyu YU ; Bo Ram LEE ; Soo Joo LEE ; Gun Sei OH

Journal of Clinical Neurology.2006;2(2):141-145. doi:10.3988/jcn.2006.2.2.141

We report unusual MRI findings (including those from diffusion-weighted imaging (DWI)) in a patient with recurrent Wernicke's encephalopathy with a remarkable cerebellar lesion. DWI showed high signal intensities in the superior portion of the cerebellar hemisphere and vermis area. After thiamine administration, clinical symptoms improved and the lesions with high signal intensities disappeared on follow-up DWI.
Cerebellum ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging* ; Thiamine ; Wernicke Encephalopathy*

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Cerebral Venous Thrombosis and Livedo Reticularis in a Case with MTHFR 677TT Homozygote.

Jee Young LEE ; Manho KIM

Journal of Clinical Neurology.2006;2(2):137-140. doi:10.3988/jcn.2006.2.2.137

Hyperhomocysteinemia associated with methylene terahydrofolate reductase (MTHFR) mutation can be a risk factor for idiopathic cerebral venous thrombosis. We describe the first case of MTHFR 677TT homozygote with cerebral venous thrombosis and livedo reticularis. A 45-year-old man presented with seizures and mottled-like skin lesions, that were aggravated by cold temperature. Hemorrhagic infarct in the right frontoparietal area with superior sagittal sinus thrombosis was observed. He had hyperhomocysteinemia, low plasma folate level, and MTHFR 677TT homozygote genotype, which might be associated with livedo reticularis and increase the risk for cerebral venous thrombosis.
Cold Temperature ; Folic Acid ; Genotype ; Homozygote* ; Humans ; Hyperhomocysteinemia ; Livedo Reticularis* ; Methylenetetrahydrofolate Reductase (NADPH2) ; Middle Aged ; Oxidoreductases ; Plasma ; Risk Factors ; Seizures ; Skin ; Superior Sagittal Sinus ; Thrombosis ; Venous Thrombosis*

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Ocular Motor Apraxia after Sequential Bilateral Striatal Infarctions.

Pil Wook CHUNG ; Heui Soo MOON ; Hwa Suk SONG ; Yong Bum KIM

Journal of Clinical Neurology.2006;2(2):134-136. doi:10.3988/jcn.2006.2.2.134

Ocular motor apraxia has been reported in bilateral frontoparietal lesions. We report a case of ocular motor apraxia after bilateral striatal infarctions. The patient had impaired voluntary saccades and smooth pursuits in the vertical and horizontal planes with an intact vestibulo-ocular reflex. Magnetic resonance imaging showed an old left putaminal infarction and an acute infarction in the right caudoputaminal area. This case demonstrates that ocular motor apraxia may occur in bilateral basal ganglia lesions.
Apraxias* ; Basal Ganglia ; Humans ; Infarction* ; Magnetic Resonance Imaging ; Pursuit, Smooth ; Reflex, Vestibulo-Ocular ; Saccades

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Cognitive Effects of Low-dose Topiramate Compared with Oxcarbazepine in Epilepsy Patients.

Sun Young KIM ; Ho Won LEE ; Doo Kyo JUNG ; Chung Kyu SUH ; Sung Pa PARK

Journal of Clinical Neurology.2006;2(2):126-133. doi:10.3988/jcn.2006.2.2.126

BACKGROUND AND PURPOSE: Low-dose topiramate (TPM) monotherapy has recently been found effective for seizure control in newly diagnosed epilepsy. In higher dosages, TPM has been associated with relatively high rates of adverse cognitive effects; similar side effects have been seen after rapid titration or polytherapy. However, its cognitive effects during low-dose monotherapy have not been established. We evaluated the cognitive effects of low-dose TPM compared with oxcarbazepine (OXC), a drug that does not appear to affect cognitive function. METHODS: Cognitive tests and subjective complaints of 30 patients with low-dose TPM monotherapy (50-200 mg/day) were retrospectively compared with those of 30 patients with OXC monotherapy at 1 year of medication. The two groups did not differ with respect to epilepsy-relevant variables, nor on baseline neuropsychological tests. RESULTS: The TPM group showed a significant difference in the performance of delayed word recall (P<0.05), backward digit span (P<0.01), and verbal fluency (P<0.05) compared with the OXC group. The TPM group showed worse performances of digit span and verbal fluency. The OXC group showed better performances of delayed word recall. The incidence of cognitive complaints was higher in the TPM group (50%) than in the OXC group (20%) (P<0.05). These cognitive effects shown in the TPM group were dose-related. The cognitive dysfunction was trivial with patients taking 50 mg/day TPM. CONCLUSIONS: Even at low-dose, TPM has a negative effect on working memory and verbal fluency compared with OXC. It can be demonstrated at 1 year of treatment.
Cognition ; Epilepsy* ; Humans ; Incidence ; Memory, Short-Term ; Neuropsychological Tests ; Retrospective Studies ; Seizures

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Clinico-electrical Characteristics of Lateral Temporal Lobe Epilepsy; Anterior and Posterior Lateral Temporal Lobe Epilepsy.

Seo Young LEE ; Sang Kun LEE ; Chang Ho YUN ; Kwang Ki KIM ; Chun Kee CHUNG

Journal of Clinical Neurology.2006;2(2):118-125. doi:10.3988/jcn.2006.2.2.118

BACKGROUND AND PURPOSE: This study aimed to determine whether there are clinicoelectrical differences between anterior lateral temporal lobe epilepsy (ALTLE) and posterior lateral temporal lobe epilepsy (PLTLE), taking medial temporal lobe epilepsy (MTLE) as a reference. METHODS: We analyzed the historical information, ictal semiologies, and ictal EEGs of temporal lobe epilepsy patients with a documented favorable surgical outcome (Engel class I or II) at follow-up after more than one year. LTLE was defined when a discrete lesion on MRI or an ictal onset zone in invasive study was located outside the collateral sulcus. LTLE was further divided into ALTLE and PLTLE by reference to the line across the cerebral peduncle. Total 107 seizures of 13 ALTLE, 8 PLTLE and 21 MTLE patients were reviewed. RESULTS: Initial hypomotor symptom was frequently observed in PLTLE (P<0.001). Oroalimentary automatism (OAA) was not observed initially in PLTLE. Generalized tonic-clonic seizures occurred significantly earlier in PLTLE than in ALTLE or MTLE (P< 0.001). Ictal scalp EEG was not helpful in differentiating between ALTLE and PLTLE. CONCLUSIONS: Frequent hypomotor onset, the absence of initial oroalimentary automatism, and early generalization are characteristic findings of PLTLE, although they are insufficient to differentiate it from ALTLE or MTLE.
Automatism ; Electroencephalography ; Epilepsy, Temporal Lobe* ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Magnetic Resonance Imaging ; Scalp ; Seizures ; Tegmentum Mesencephali ; Temporal Lobe*

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Handedness and Asymmetry of Motor Skill Learning in Right-handers.

Jinwhan CHO ; Kyung Seok PARK ; Manho KIM ; Seong Ho PARK

Journal of Clinical Neurology.2006;2(2):113-117. doi:10.3988/jcn.2006.2.2.113

BACKGROUND AND PURPOSE: The most remarkable behavioral asymmetry is handedness. The preferred hand often has better performance, motor strength, nonpreferred hand. However, whether these components are associated with skill learning is not clear. METHODS: We evaluated healthy right-handers by setting a series of motor-performance tasks including skill learning, grip strength, and speed. RESULTS: The preferred hand showed better skill performance and learning rate. However, the degree of the right-left difference in grip strength or speed difference did not correlate with the asymmetry in skill-learning rate. Therefore, although the preferred hand exhibits a better skill-learning capacity than the nonpreferred hand, asymmetry in skill learning cannot be explained by motor strength or speed. CONCLUSIONS: Our findings suggest that better skill performance of the right hand in right-handers cannot be attributed to the degree of hand preference score, strength, or motor speed.
Functional Laterality* ; Hand ; Hand Strength ; Learning* ; Motor Skills*

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Diffusion Weighted Imaging Findings in the Acute Lateral Medullary Infarction.

Min Jung SEO ; Sook Young ROH ; Yu Suck KYUN ; Hyun Jung YU ; Young Kuk CHO

Journal of Clinical Neurology.2006;2(2):107-112. doi:10.3988/jcn.2006.2.2.107

BACKGROUND AND PURPOSE: Negative findings on diffusion-weighted imaging (DWI) does not exclude the possibility of brainstem infarction, particularly in the acute stage of medullary lesion. Our aim was to investigate the false-negative rate of DWI in patients with acute lateral medullary infarction. METHODS: We applied DWI to 26 patients with a clinical diagnosis of lateral medullary infarction within 72 h of the onset. We assessed relationships between initial DWI findings and time-to-MRI (the time between onset of symptoms and initial DWI), number of clinical symptoms and signs, and final lesion volume. RESULTS: There were 8 cases (31%) of false negatives in the initial DWI results. The occurrence of false-negative DWI findings decreased significantly as the time-to-MRI increased (P=0.014). However, the false-negative rate was not significantly correlated with the number of clinical symptoms and signs or the final lesion volume. CONCLUSIONS: The diagnosis of lateral medullary infarction should not be ruled out on the basis of early negative DWI. To confirm the lesion, follow-up DWI or further MRI should be performed in cases with early negative DWI results
Brain Stem Infarctions ; Diagnosis ; Diffusion* ; Follow-Up Studies ; Humans ; Infarction* ; Magnetic Resonance Imaging

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Charcot-Marie-Tooth Disease: Seventeen Causative Genes.

Jung Hwa LEE ; Byung Ok CHOI

Journal of Clinical Neurology.2006;2(2):92-106. doi:10.3988/jcn.2006.2.2.92

Charcot-Marie-Tooth disease (CMT) is the most common form of inherited motor and sensory neuropathy. Moreover, CMT is a genetically heterogeneous disorder of the peripheral nervous system, with many genes identified as CMT-causative. CMT has two usual classifications: type 1, the demyelinating form (CMT1); and type 2, the axonal form (CMT2). In addition, patients are classified as CMTX if they have an X-linked inheritance pattern and CMT4 if the inheritance pattern is autosomal recessive. A large amount of new information on the genetic causes of CMT has become available, and mutations causing it have been associated with more than 17 different genes and 25 chromosomal loci. Advances in our understanding of the molecular basis of CMT have revealed an enormous diversity in genetic mechanisms, despite a clinical entity that is relatively uniform in presentation. In addition, recent encouraging studies - shown in CMT1A animal models - concerning the therapeutic effects of certain chemicals have been published; these suggest potential therapies for the most common form of CMT, CMT1A. This review focuses on the inherited motor and sensory neuropathy subgroup for which there has been an explosion of new molecular genetic information over the past decade.
Axons ; Charcot-Marie-Tooth Disease* ; Classification ; Explosions ; Genes, X-Linked ; Humans ; Inheritance Patterns ; Models, Animal ; Molecular Biology ; Peripheral Nervous System

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Syndromic Approach to Parkinson's Disease: Role of Functional Imaging.

Chong Sik LEE ; Sun Ju CHUNG ; Jae Seung KIM

Journal of Clinical Neurology.2006;2(2):83-91. doi:10.3988/jcn.2006.2.2.83

Current evidence from monogenic Parkinson's disease (PD) supports the view that PD is a clinical syndrome, rather than a single disease entity, and that the heterogeneity of PD indeed reflects different pathogenesis. Recent developments in functional imaging have enabled the in vivo assessment of cellular and molecular pathology of PD with respect to temporal and topographical patterns. We propose that this new technology will be useful for linking monogenic and sporadic PD, and thus, for classifying PD based on the pathogenesis. It will be also useful in clinico-genetic studies exploring susceptibility factors and at-risk groups, which are important for neuroprotective treatment when it becomes available.
Parkinson Disease* ; Pathology ; Pathology, Molecular ; Population Characteristics ; Positron-Emission Tomography

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