Leprous Neuropathy Associated with Predominant Proprioceptive Sensory Loss / 대한임상신경생리학회지

Some patients with leprosy may present with atypical features, such as isolated peripheral neuropathy without skin lesions, or marked proprioceptive dysfunction. We report a 56-year-old female who presented with predominant proprioceptive loss without skin lesion, but was finally confirmed as leprous neuropathy by sural nerve biopsy. It is postulated that large myelinated fibers were affected by chronic immunological reactions triggered by inactive bacterial particles, producing a peripheral neuropathy presenting as predominant proprioceptive sensory loss without typical skin lesions.

Clinical and Pathological Features of Clevudine Induced Myopathy

BACKGROUND: Clevudine (Revovir(R)) is a recently introduced antiviral drug, and clinical trials have demonstrated its potent, sustained antiviral activity without specific adverse events. However, several studies have found severe myopathy during clevudine therapy. Our study aimed to summarize the clinical and pathological features of clevudine-induced myopathy. METHODS: We analyzed the demographic data, clinical features, and pathologic findings of 18 consecutive hepatitis-B patients who developed skeletal myopathy during clevudine therapy. RESULTS: The 18 patients comprised 11 women and 7 men aged 48.2+/-14.0 years (mean+/-standard deviation; range 28-74 years). Each of the 18 patients was treated with clevudine for at least 5 months (range 5-20 months) before the development of symptoms. In all patients the main symptom was proximal muscular weakness that progressed slowly over several months. Elevated creatine kinase and myopathic patterns on electromyography were found. Muscle biopsies revealed severe myonecrosis associated with numerous ragged red fibers and cytochrome-c-oxidase-negative fibers, mitochondrial proliferation, and predominant type-II fiber atrophy. The muscle weakness gradually improved within 20 weeks after discontinuation of clevudine. CONCLUSIONS: Clevudine therapy can induce myopathy associated with mitochondrial toxicity. Careful clinical and laboratory monitoring of the skeletal muscle dysfunction is required in patients receiving clevudine therapy.

A Case of Gerstmann-Straussler-Scheinker Disease

BACKGROUND: Gerstmann-Straussler-Scheinker disease (GSS) is a type of human transmissible spongiform encephalopathy (TSE) that is determined genetically. CASE REPORT: A 46-year-old woman presented with a slowly progressive ataxic gait and cognitive decline. She was alert but did not cooperate well due to severe dementia and dysarthria. High signal intensities in the cerebral cortices were evident in MRI, especially in diffusion-weighted images (DWI). A prion protein gene (PRNP) analysis revealed a P102L (proline-to-leucine) mutation in codon 102. CONCLUSIONS: This is the first reported case of GSS (confirmed by PRNP analysis) in Korea. Distinctive MRI findings are also presented.

ERRATUM: Cognitive Impairment in Parkinson's Disease without Dementia: Subtypes and Influences of Age

The publisher wishes to apologize for incorrectly displaying the author (Seong Yeon Kim)' name, academic degree and position. We correct his name from Seong Yeon Kim to Seongyeon Kim and his academic degree from MD to PhD. He belongs to the division of Management Information Science, College of Business Administration, Dong-A University, Busan, Korea

Cognitive Impairment in Parkinson's Disease without Dementia: Subtypes and Influences of Age

BACKGROUND AND PURPOSE: Cognitive impairments are common in Parkinson's disease (PD), although the severity of these impairments does not significantly impair the patient's daily activities. The aim of this study was to determine the frequency of mild cognitive impairment (MCI) of Parkinson's disease (PDMCI) and its subtypes in nondemented PD patients. We also evaluated the influence of age on the pattern of subtypes of PDMCI. METHODS: A total of 141 consecutive, nondemented PD patients underwent a comprehensive neuropsychological assessment covering the five cognitive domains: attention, language, visuospatial, memory, and executive functions. PDMCI was defined as impaired performance in at least one of these five cognitive domains. The influence of age on the distribution of subtypes of PDMCI was assessed by comparing patients in two groups dichotomized according to their age at assessment (younger vs. older). RESULTS: Fifty-seven (40.4%) of the nondemented PD patients had an impairment in at least one domain, and were therefore considered as having PDMCI. The age at assessment and age at disease onset were significantly higher in the PDMCI patients. The amnestic type of PDMCI was the most frequent, followed by the visuospatial, linguistic, executive, and attention types in that order. The frequency of PDMCI was higher for all subtypes in the older group; the domain that was influenced the most by age was executive function. CONCLUSIONS: MCI was common in PD and the subtypes were diverse. Age was found to be an important risk factor for the development of PDMCI, particularly for the executive subtype. These results indicate that the concept of MCI should be introduced in PD.

The Changes of High Sensitive C-reactive Protein by Atorvastatin 20 mg in Acute Ischemic Stroke

BACKGROUND: It has been reported that early prescription of statin might enhance the vascular protection in acute atherothrombosis by its various mechanisms. However, until now, there has been little information about the serial changes of high sensitive C-reactive protein (hs-CRP), one of the notable inflammatory markers in atherothrombosis, by early prescription of statin in acute ischemic stroke. METHODS: We retrospectively collected one hundred eighteen (118) patients with acute ischemic stroke who had conformed to the Atherosclerotic Stroke Mechanism Algorithm (ASMA), one of the guidelines for statin, and evaluated the effects of Atorvastatin 20 mg on the changes of hs-CRP levels and clinical severity at 7 days and 30 days after ischemic events. RESULTS: Among the 118 patients who should have been prescribed statin on the ASMA guideline, sixty-three patients (53.4%) used the statin (Atorvastatin 20 mg) within 48 hrs after admission. Serum concentration of hs-CRP levels was decreased from admission to 30 days later in both statin and non-statin users. Particularly, its extent was significant (0.39+/-0.74 vs 0.75+/-0.98 mg%, p=0.042) after 30 days of ischemic events in statin users. Statin users had lower mortality and vascular recurrence (p=0.083) and fewer bad prognosis rates (27.7% vs. 34.5%) compared to non-statin users over 30 dyas after ischemic events, although there was no statistical significance. CONCLUSIONS: These results suggest that early prescription of statin (Atorvastatin 20 mg) might regulate the inflammatory activity over 30 days after acute ischemic stroke.

A Case of Chrug-Strauss Syndrome Complicated with Intestinal Perforation

Gastrointestinal symptoms of Churg-Strauss syndrome (CSS) are common, but intestinal perforation is very unusual and occurred during steroid therapy in the previous case. We report a CSS showing intestinal perforation, which was caused by a complication of vasculitis.

A Case of Bacterial Meningitis Associated with Cerebral Infarction and Arterial Stenosis-Transcranial Doppler Findings

In spite of the appropriate antibiotic treatment and the development of newer antibiotics, bacterial meningitis still has a high risk of complications. Especially, vascular involvements of meningitis, including vasospasms and infarctions, frequently result in neurologic sequelae. Here, we report a case of bacterial meningitis complicated by arterial vasospasms and multiple infarctions during a clinically improving course. This case suggests that in the management of bacterial meningitis, early evaluation and monitoring of vasculitis by TCD and its management could prevent fatal outcomes.

A Case of Propofol-Induced Delayed-Onset Refractory Myoclonic Seizures

Propofol, a GABA-mediated inhibitor of excitatory neurotransmitter, is a popular intravenous agent for general anesthesia and sedation. Its side effects reportedly include opisthotonus, seizures, and myoclonus, and are usually manageable. We present a patient who developed propofol-induced delayed-onset refractory myoclonic seizures that resisted antiepileptic drugs.

A Case of Propofol-Induced Delayed-Onset Refractory Myoclonic Seizures

Propofol, a GABA-mediated inhibitor of excitatory neurotransmitter, is a popular intravenous agent for general anesthesia and sedation. Its side effects reportedly include opisthotonus, seizures, and myoclonus, and are usually manageable. We present a patient who developed propofol-induced delayed-onset refractory myoclonic seizures that resisted antiepileptic drugs.

Reflex Epilepsy Induced by Tooth Brushing: A Case Report / 대한간질학회지

Reflex epilepsy describes seizures which are precipitated by clearly recognized stimuli. Reflex epileptic seizures triggered by tooth brushing are rare. We report a case with reflex epilepsy occurring exclusively during tooth brushing. He began having seizures at age 28. Neurological examinations and brain magnetic resonance images were found normal. On long term video-EEG monitoring, he presented two episodes of simple partial seizure induced by brushing molar and premolar teeth, which was right facial clonic movement persisting 3-5 seconds. He was unresponsive during the event, but had no postictal confusion. Ictal EEG showed 6 Hz rhythmic theta activity in the left frontocentral area. Interictal EEG was normal. The findings of EEG suggest that this reflex epilepsy might have epileptic focus in the left frontocentral area. Reasonable precaution such as gargling or less vigorous brushing of his teeth with fingers effectively prevented seizure recurrence.