Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy

Background@#and Purpose To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured antiHMGCR antibodies and analyzed the clinical, radiological, and pathological features of patients with anti-HMGCR myopathy. @*Methods@#We measured titers of anti-HMGCR antibodies in the sera of 99 patients with inflammatory myopathy, 36 patients with genetic myopathy, and 63 healthy subjects using an enzyme-linked immunosorbent assay. We tested 16 myositis-specific autoantibodies (MSAs) in all patients with anti-HMGCR myopathy. @*Results@#Positivity for the anti-HMGCR antibody was observed in 17 (4 males and 13 females) of 99 patients with inflammatory myopathy. The median age at symptom onset was 60 years.Ten (59%) of the patients with anti-HMGCR positivity had taken statins. The titer of antiHMGCR antibodies was significantly higher in the statin-naïve group (median=230 U/mL, interquartile range=170–443 U/mL) than in the statin-exposed group (median=178 U/mL, interquartile range=105–210 U/mL, p=0.045). The most common symptom was proximal muscle weakness in 15 patients (88%), followed by myalgia in 9 (53%), neck weakness in 4 (24%), dysphagia in 3 (18%), and skin lesions in 2 (12%). The median titer of anti-HMGCR antibody was 202 U/mL. We found eight different MSAs in nine (53%) patients. The median disease duration from symptom onset to diagnosis was significantly shorter in the MSA-positive group than in the MSA-negative group (p=0.027). @*Conclusions@#Our study was the first to measure anti-HMGCR antibodies in inflammatory myopathy. It has provided new findings, including the suggestion of the coexistence of other MSAs in Korean patients.

Pneumoncoccal Meningitis Presenting with Ventriculitis and Pneumocephalus

No abstract available.

Clebopride-Induced Parkinsonism

No abstract available.

Atypical Anterior Spinal Artery Infarction due to Left Vertebral Artery Occlusion Presenting with Bilateral Hand Weakness

BACKGROUND: Infarct of the anterior spinal artery is the most common subtype of spinal cord infarct, and is characterized by bilateral motor deficits with spinothalamic sensory deficits. We experienced a case with atypical anterior-spinal-artery infarct that presented with bilateral hand weakness but without sensory deficits. CASE REPORT: A 29-year-old man presented with sudden neck pain and bilateral weakness of the hands. Magnetic resonance imaging (MRI) of the brain did not reveal any lesion. His motor symptoms improved rapidly except for mild weakness in his left wrist and fingers. Magnetic resonance angiography showed proximal occlusion of the left vertebral artery; a spine MRI revealed left cervical cord infarction. CONCLUSIONS: Bilateral or unilateral hand weakness can be the sole symptom of a cervical cord infarct.

Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.

Multiple Cerebral Infarcts Following Acute Plasmodium vivax Infection / 대한뇌졸중학회지

Cerebral malaria is a severe neurological complication of Plasmodium falciparum infection. Cerebral malaria can lead to cerebral infarction by several mechanisms including systemic inflammatory response. The systemic inflammatory response is known to rarely occur in Plasmodium vivax infection. We report a patient who developed multiple cerebral infarctions following Plasmodium vivax infection.

Sequential Involvement of the Nervous System in Subacute Combined Degeneration

PURPOSE: Subacute combined degeneration (SCD) involves progressive degeneration of the spinal cord, optic nerve, and peripheral nerves. Vitamin B12 (VB12) is a co-factor in myelin synthesis. Because each cell that constitutes the myelin component in the central nervous system and peripheral nervous system is different, it is improbable that these cells undergo simultaneous degeneration. However, the sequence of degeneration in SCD has not been established. MATERIALS AND METHODS: In this study, we analysed medical records and electrophysiological data of patients who showed neurological symptoms and whose serum VB12 levels were lower than 200 pg/mL. RESULTS: We enrolled 49 patients in this study. Their mean VB12 level was 68.3 pg/mL. Somatosensory evoked potential (SEP) study showed abnormal findings in 38 patients. Of the 40 patients who underwent visual evoked potential (VEP) study, 14 showed abnormal responses. Eighteen patients showed abnormal findings on a nerve conduction study (NCS). In this study, abnormal posterior tibial nerve SEPs only were seen in 16 patients, median nerve SEPs only were seen in 3 patients, abnormal VEPs only in two, and abnormal NCS responses in one patient. No patient complained of cognitive symptoms. CONCLUSION: In SCD, degeneration appears to progress in the following order: lower spinal cord, cervical spinal cord, peripheral nerve/optic nerve, and finally, the brain.

Erratum: Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

Multiple Ischemic Stroke as an Initial Manifestation of a Concealed Pancreatic Adenocarcinoma

No abstract available.

Sacral Herpes Simplex Infection Mimicking as Herpes Zoster in the Immune-Competent Patient

No abstract available.

Acute Inflammatory Facial Nerve Paralysis / 대한이비인후과학회지

Bell's palsy is the most common form of acute facial nerve disorder, which presents as an acute peripheral unilateral facial palsy of unknown cause. Ramsay-Hunt syndrome is the second most common cause of acute facial palsy, and is known to be caused by reactivation of latent varicella zoster virus. The main goal of treatment for acute inflammatory facial nerve paralysis is to speed up recovery, to facilitate the recovery more completely and to prevent other sequelae. However, some patients may have a poor recovery with permanent, disfiguring facial asymmetry despite of many kinds of treatments. Regarding the diagnostic and therapeutic issues of the two common disorders, there still exist some controversies. This article reviewed recent evidences on several important issues in evaluation and management of acute inflammatory facial nerve paralysis, and intended to provide an evidence-based framework for decision-making in the clinic.

A Case With Suspected Tick Paralysis

Tick paralysis is caused by a neurotoxin secreted by female tick. Characteristic initial manifestation is bilateral flaccid ascending paralysis similar to Guillain-Barr? syndrome. The predominant electrophysiological abnormality is a reduction in complex muscle action potentials. Here, we present a 62-year-old man who initially experienced a sudden biting pain on his scalp. Subsequently he developed bilateral lower extremity paralysis that ascended symmetrically involving the upper extremities. Within 2 weeks, the patient showed a full recovery without treatment.

Two Cases of Delayed Post-radiation Lower Cranial Nerve Palsies

Radiation-induced lower cranial neuropathy shows a clinical presentation similar to tumor recurrence or amyotrophic lateral sclerosis. We experienced two patients with bulbar palsies several years after radiotherapy for nasopharyngeal cancer. Brain magnetic resonance imaging showed no evidence of tumor recurrence. Electrophysiologic studies demonstrated mild denervation changes and myokymic discharges in muscles innervated by cranial nerves. Bulbar palsies progressed for 1 year then became stable. We emphasize the importance of myokymic discharges in the differential diagnosis of radiation-induced cranial neuropathy as radiation plexopathies.

Long-Term Outcomes of Acute Ischemic Stroke in Patients Aged 80 Years and Older

PURPOSE: Short life expectancy influences decision-making when treating very old patients with acute ischemic stroke (AIS). We investigated mortality and survival duration in very old AIS patients (> or =80 years) who received hospital care. PATIENTS AND METHODS: Mortality data were obtained from medical records, structured telephone inquiries, death certificates from the Korean National Statistical Office, and social security data 5+/-1.9 years after stroke onset. Age, gender, vascular risk factors, and functional outcomes from modified Rankin scales (MRS) at discharge were analyzed as predictors of mortality. RESULTS: Among 134 patients, 92 (68.7%) died. On Kaplan- Meier analysis, duration of survival of patients aged 80-84 years was longer than those aged 85-89 or 90-94 (24+/-6.4, 8+/-7.3, 7+/-2.0 months, respectively, p=0.002). Duration of survival of patients discharged in a state of MRS 0-1 was longer than the remaining groups at 47+/-4.8 months (p<0.001). In Cox proportional hazard analysis, age and MRS at discharge were independent predictors of mortality. CONCLUSION: Long-term outcomes of very old patients with AIS are not uniformly grave, therefore predictors of mortality and estimated duration of survival should be considered during decision- making for treatment.

Features of the Myelopathy in Patients with Electrical Burn

BACKGROUND: Electrical injury can cause serious damage to any part of the nervous system. However spinal cord injury by electricity itself rarely develops. If develops, it can be either electrical myelopathy (immediate or delayed), spinal atrophic paralysis and/or amyotrophic lateral sclerosis-like disease. We are going to report the clinical, electrophysiological and radiographic features of electrical myelopathy (immediate or delayed) of 18 patients. METHODS: We retrospectively reviewed the clinical, electrophysiological and radiographic data of patients who were diagnosed as electrical myelopathy. RESULTS: Among 1,306 patients with electrical injury, 18 patients (1.4%) had electrical myelopathy. Fifteen patients (83%) had motor symptoms and 16 patients (89%) had sensory symptoms or signs. It is interesting for seven patients to appear their neurological symptoms or signs with a day after electrical injury. Somatosensory evoked potentials (SEP) which were done in 17 patients showed abnormal central conduction defects in 12 patients (70.5%). Magnetic resonance images (MRI) of spinal cord, however, did not show any abnormalities in all the tested patients (15 patients). CONCLUSIONS: Clinically, electrical myelopathy can cause not only motor but also sensory symptoms or signs. Interestingly, there were several immediate as well as delayed forms of electrical myelopathy In most patients with electrical myelopathy, SEP was a useful method to detect objective abnormalities but MRI was not.

Reversible Diffuse Brain Edema in Acute Encephalopathy with Shigellosis

Shigellosis is an acute inflammatory colitis by infection to one of the members of the genus Shigella. It is known that various CNS symptoms including headache and seizure can develop with shigellosis. Encephalopathy with severe brain edema in shigellosis were rarely reported even in children as well as in adults. We report a rare case of acute encephalopathy with shigellosis showing severe diffuse brain edema which resolved rapidly with administration of steroids in an adult.

Electrophysiological Tests in Generalized Tetanus

BACKGROUND: Tetanus toxin selectively blocks inhibitory synapses in the brainstem as well as the spinal cord. Therefore, in contradiction to Stiff Person syndrome, patients with generalized tetanus usually show abnormal masseter silent periods as well as abnormal F/M amplitude or H/M amplitude ratios. This study aimed to verify the characteristics of electrophysiological findings of generalized tetanus. METHODS: The authors retrospectively studied clinical and electrophysiological characteristics of 7 patients with generalized tetanus, who were admitted to the neurology department of Hallym Medical Center from 1995 to 2005. RESULTS: All the seven patients showed abnormal masseter silent periods. Three of them showed somewhat improvement in the silent period at follow-up study as trismus was improving. Full NCSs done in two patients did not show any abnormalities except an increased F/M amplitude ratio. One patient with a wound site in his left finger showed an abnormal F/M amplitude ratio only in the right upper extremity without involvement of other extremities. Another patient showed an increased H/M amplitude ratio without an increased F/M amplitude ratio. (In this patient we did not conduct full NCS tests.) CONCLUSIONS: The Masseter silent period could be used as a diagnostic tool and parameter of clinical improvement in patients with generalized tetanus.

Complete Binocular Blindness as the First Manifestation of HIV-Related Cryptococcal Meningitis

Ocular complications of HIV-related cryptococcal meningitis are reasonably common, but complete binocular blindness as the first manifestation of HIV is extremely rare. A 58-year-old man presented with binocular blindness. He experienced blurred vision for 3 days before the blindness. Mild pleocytosis was present in the cerebrospinal fluid, from which Cryptococcus neoformans was cultured. Serology revealed positivity for HIV antibody. He was treated with antifungal and antiretroviral therapy. This case indicates that HIV-related cryptococcal meningitis should be taken into consideration when determining the cause of unexpected sudden binocular blindness.

A Case of Neurologic Symptom in Association with Intoxication after Ingestion of Codfish Intestine

No abstract available.

Delayed-Onset Continuous Bruxism with Olivary Hypertrophy After Top of the Basilar Syndrome

Delayed-onset continuous bruxism due to brain stem infarction has not yet been reported. A 49-year old man presented with quadriplegia and ophthalmoplegia. Brain MRI showed acute infarction in the bilateral midbrain, right thalamus and the superior cerebellum. One month later, the patient developed bruxism which persisted during sleep. A palatal myoclonus was not observed. Follow up MRI taken 4 months later showed bilateral olivary hypertrophy. We suggest that the patient's bruxism may be related to the olivary hypertrophy. The bruxism generator may be located in the pontine-reticular-formation (PRF). Bilateral large midbrain lesions interrupting the cortical inhibition may have produced bilateral olivary hypertrophy, which could stimulate the PRF, producing continuous bruxism.