Acta neurologica Taiwanica最新文献

Objective: Macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) refers to a set of clinical symptoms caused by the excessive activation and proliferation of macrophages. It was linked with autoimmune disease such as systemic-onset juvenile rheumatoid arthritis, systemic lupus erythematosus, rheumatoid arthritis, and dermatomyositis, etc. Herein we report a case of myasthenia gravis (MG) with concurrent cytomegalovirus (CMV) infection developed MAS.

Case report: A 31-year-old female with history of MG for 2 years under stable control with azathioprine and prednisolone. She presented with persistent high fever for 2 weeks after an upper respiratory infection. Lab data revealed pancytopenia, elevated triglyceride, ferritin and C-reactive protein (CRP). A bone marrow aspiration confirmed hemophagocytosis. Investigation for occult infection revealed her plasma was positive for CMV IgG and IgM, and high for CMV viral load. She was then treated with 5 sessions of plasmapheresis and pulse steroid. Azathioprine was discontinued and replaced with cyclosporine. Gancylovir was given for her concurrent CMV infection. After 2 weeks of treatment, her fever gradually subsided, and her blood cell count, hepatobiliary enzymes, ferritin and CRP have returned to normal range. She was discharged in good recovery.

Conclusion: MAS is a rare complication of systemic autoimmune disease with poor prognosis, which may be precipitated by concurrent infection. Early recognition of this syndrome and prompt immune modulation therapy is crucial for successful treatment.

Purpose: The patients with more severe stroke, have more chance to develop higher levels of cognitive impairments; and family history of dementia as a genetic background, can give rise to an increased risk of the severity of cognitive deterioration. In this study, we sought to investigate whether the risk alleles of Val66Met of brain-derived neurotrophic factor (BDNF) polymorphism, has a destructive interaction with the stroke severity (SS) and family history of dementia (FHD) for cognitive impairments?

Method: In a case-control study, the carriers of at least one Val allele (n=56) were compared to the carriers of Met/Met homozygotes (n=156) in terms of FHD and SS (through National Institutes of Health Stroke Scale) on the north of Iran. To determine the cognitive functions, the third version of Addenbrooke's Cognitive Examination (ACE-III) was used.

Result: The mean age of patients was 64.52±11.71, and in average 202 day had passed from their stroke. The interactive effects of genotypes Val66Met BDNF with SS[F=8.95, ή2=0.04, P=0.003] and FHD[F=4.59, ή2=0.02, P=0.03] were significant for total score of ACE-III. It means that the Met/ Met homozygosity, modulated the effect of risk factors of SS and FHD on the cognitive function. Such homozygosity protects the attentional function and language abilities against the SS and FHD(P≤0.05).

Conclusion: It can be speculated that presence of Val/Met heterozygosity has a destructive interaction with the SS and FHD for decreasing the cognitive function, particularly in attention and language domains. Our findings suggested that the inhibition of signaling and trafficking of Val/Met heterozygosity is possibly a practical strategy in reducing the cognitive impairments following the stroke.

Purpose: Focal signs are a big deal in neurology and are among the most important clues leading to diagnosis and localization. Wernekink commissure syndrome is due to lesions in the caudal paramedian midbrain involving the entire decussation of the superior cerebellar peduncles, resulting in the clinical hallmark of a bilateral cerebellar syndrome.

Case report: A 79-year-old man presented with sudden, severe unsteadiness associated with slurring of speech, binocular double vision, and bilateral hand tremor. Examination showed right INO, moderately severe dysarthria, bilateral dysmetria and dysdiadochokinesia, with severe truncal ataxia and bilateral upper and lower limb ataxia. Also, bilateral coarse tremor was noted in both hands which was present at rest, action and on reaching for objects. Brain MRI revealed an acute infarction involving the Wernekinck decussation in the right caudal midbrain and mesencephalo-pontine junction.

Conclusion: The differential of Wernekink Commissure Syndrome is complex, and localization and lateralization are extremely difficult owing to prominent bilateral cerebellar symptoms. The finding of an associated unilateral INO in some cases makes it possible to confidently narrow the list of differentials and localize the lesion to the paramedian tegmentum ipsilateral to the non-adducting eye.

Purpose: A case report with a review of the current literature concerning cutaneous necrosis has occasionally been reported in interferon therapy.

Case report: We report a 19-year-old woman diagnosed multiple sclerosis for three years. She selfinjected the standard dose of recombinant interferonβ-1a (12 million units) subcutaneously three times a week. Severe necrotizing cutaneous reactions over abdomen Happened and she must receive parental antibiotics and surgical debridement.

Conclusion: Our observation emphasizes the importance of educating patients on the proper selfadministration of subcutaneous injections of interferon β.

Purpose: MR perfusion weighted imaging (PWI) has been used as sensitive indicator of tissue at risk for infarction. Quantitative perfusion parameters such as cerebral blood flow (CBF), mean transit time (MTT) and cerebral blood volume (CBV) can be obtained from post processing of PWI data using standard singular value decomposition algorithm (SVD). Assumption regarding absence of arterial - tissue delay (ATD) used in SVD algorithm results in underestimation of perfusion parameters. To estimate accurate values for perfusion parameters it is important to understand the mathematical framework behind SVD and improved SVD algorithms (bSVD and rSVD).

Method: This study explains the mathematical framework of SVD and improved SVD algorithms and uses computational techniques that use bSVD algorithm to obtain perfusion parameters maps of CBF, CBV and MTT for acute stroke patient.

Result: Computational techniques based on mathematical deconvolution algorithms are used to post process CBV, CBF and MTT maps where decrease in CBF and CBV were seen in left hemisphere.

Conclusion: The bSVD algorithm is found to be sensitive to ATD and provides more accurate estimates of perfusion parameters than the SVD algorithm, however CBF estimates from bSVD and rSVD still remain influenced by other artifacts Keywords: PWI = perfusion weighted imaging, CBF= cerebral blood flow, MTT = mean transit time, CBV= cerebral blood volume, SVD = singular value decomposition algorithm.

Purpose: Spinal infarction is a rare condition and usually presents with a sudden or acute course. A prolonged course is rare and may mimic the presentation of inflammatory myelitis. Here we present a case of atypical spinal cord infarction with a stuttering course for six days..

Case report: A 47-year-old male presented initially with symptoms of sudden onset, limb pain. Sudden chest pain radiating to the back, occurred three days later. Sudden urinary retention and quadriparesis were presented after another three days. The diagnosis of spinal cord infarction was made through diffusion restriction in spinal magnetic resonance imaging.

Conclusion: A prolonged course of spinal cord infarction is relatively uncommon but a stepwise and stuttering course may provide clues. Diffusion restriction in magnetic resonance imaging also may be helpful. The diagnosis of spinal cord infarction should always be kept in mind.

Purpose: This study was designed to investigate the effect of apilarnil on neuronal damage and related mechanisms in a sepsis model in order to demonstrate whether or not apilarnil has neuroprotective effect.

Methods: In this study, 64 adult male Sprague-Dawley species rats were randomly divided into eight groups. The rats were administered apilarnil and/or lipopolysaccharide (LPS). Superoxide dismutase (SOD), catalase (CAT), malondialdehyde (MDA), xanthine oxidase (XOD) and testican-1 levels were measured in the brain tissue. Proinflammatory cytokines (tumor necrosis factor alpha [TNF-α], interleukin 1 beta [IL-1β], interleukin 6 [IL-6]) were measured in brain tissue. Histological examinations were performed on hippocampus and cortex tissues in all groups. Apoptotic cell count was estimated using the Tunel method to observe the apilarnil's effect on apoptosis. Purkinje cells were counted in the hippocampus to measure the protective effect of apilarnil on the hippocampus.

Results: Apilarnil reduced the decrease in SOD and CAT levels in the brain developing sepsis. Apilarnil reduced the increase in MDA, XOD, and testican-1 levels in the septic brain. It was observed that the number of degenerated neurons due to sepsis decreased as apilarnil dose increased. Apilarnil reduced the elevated levels of proinflammatory cytokines (IL-6, TNF-α, IL-1β) induced by sepsis. Apilarnil prevented sepsis-related apoptosis in the brain.

Conclusion: The neuroprotective potential of apilarnil against brain damage in the sepsis model was demonstrated and suggested that it has the potential to contribute to new therapeutic targets against various neurological disorders.

Purpose: To study the role of early serial EEGs in improving seizure freedom rates after initiation of ethosuximide or valproic acid for childhood absence epilepsy.

Methods: Retrospective data analysis study of AED naive patients with childhood absence epilepsy undergoing treatment at the community-based epilepsy clinic. Due to small sample size Fisher's exact test was used to determine two-tailed p value at less than 0.05 statistical significance..

Results: At 2-month study period 71.4% patients in the ethosuximide and 87.5% in the valproic acid group achieved seizure freedom, with EEG normalization in 21.4% and 50% respectively. At 6-month study period, in patients continuing ethosuximide, seizure freedom and EEG normalization rates were 89.5% and 52.6% respectively; while in patients continuing valproic acid, results were 100% and 78.6% respectively. Both at 2-month and 6-month study periods, a trend towards higher seizure freedom was noted in patients with typical versus non-typical epileptiform discharges at baseline with the valproic acid group showing a superior response.

Conclusion: Although no statistically significant difference in response rates was noted, 1) a shift towards higher seizure freedom with valproic acid; 2) improved response after switching to valproic acid at 2 months, if warranted; and 3) superior response rate in patients with typical EEG epileptiform discharges at baseline were observed. A larger study is needed to define the role of early serial EEGs to delineate higher drug failure probability; and to determine the importance of non-typical EEG characteristics at baseline in relation to the choice of AED and long-term outcome Keywords: childhood absence epilepsy, ethosuximide, valproic acid, non-typical epileptiform discharges.

Facial ArterioVenous Malformations (AVM) are rare lesions and present great difficulty in their diagnosis and treatment. We report a case of a 24-year-old male who has been diagnosed a right facial AVM that underwent endovascular embolization with a liquid embolic device and consequently surgical resection. The type of liquid embolic device used has given advantage for both treatment techniques.