Acta neurologica Taiwanica最新文献

There is great interest in crosstalk between the gastrointestinal and immune systems. Small intestinal bacterial overgrowth (SIBO) is a bowel disorder prevalent among patients with Parkinson's disease; SIBO treatment has been shown to modulate neurological inflammation, motor and cognitive outcomes there. However, to date, no link between Alzheimer's dementia and SIBO has been established. This pilot study sought to estimate the prevalence of SIBO in Alzheimer's dementia in the outpatient setting in Singapore General Hospital. It entailed performing a hydrogen breath test and objectively scoring gastrointestinal symptoms and their severity in 48 patients, comparing symptom scores and mean breath test values in those with mild to moderate Alzheimer's against age- and sex-matched controls that did not fulfill DSM-V criteria for probable Alzheimer's. Here, the prevalence of positive breath tests and symptoms of SIBO were no greater among Alzheimer's patients than in controls. This suggests that the gut microbiome changes and increased bowel inflammation seen in previous studies on Alzheimer's patients are likely effected through pathways other than SIBO, and are likely more complex than a mere increase in small bowel bacterial volume. Rather, future research could be directed along the lines of qualitative changes in small bowel microbiota, or pathologies in other parts of the gastrointestinal tract such as the colon or stomach, aspects which are not adequately captured by the hydrogen breath test. Keywords: Alzheimer's disease; dementia; gut-brain axis; small intestinal bacterial overgrowth; microbiome.

Purpose: Neuromyelitis optica (NMO) spectrum disorder and multiple sclerosis (MS) have similar clinical presentations which may make a diagnostic difficulty, especially when the data of aquaporin-4 (AQP4) antibody is not available. We reported the diagnostic and therapeutic dilemma of a woman with a delayed diagnosis of NMO spectrum disorder for more than 20 years.

Case report: The patient was a 51 years old woman who suffered from several episodes of relapsing and remission of limbs weakness, visual impairment and gait disturbance since 29 years old. She was diagnosed as a case of MS and received treatment accordingly. Treatment with the use of Rebif was started since 2008-2012, and was then shifted to Fingolimod due to several minor attacks were still noted during this period. Serum AQP4-IgG was checked before the use of Fingolimod by using Enzyme-linked immunosorbent assay (ELISA) and the result showed sero-negative for this Ab. However, occasional minor attacks were still noted. In May 2018, severe relapsing developed and brain magnetic resonance imaging (MRI) showed marked progression of the brain lesion. Initially, progressive multifocal leukoencephalopathy was suspected, but both cerebrospinal fluid and serologic study for John Cunningham virus (JCV) were negative. AQP4-IgG was rechecked by using cell-based assay (CBA), and the result showed positive finding. Thereafter, her therapy was changed to NMO spectrum disorder regimen.

Conclusion: It is worthwhile to recheck the serum AQP4-IgG if the initial study showed negative result by using ELISA since CBA has higher sensitivity than previous study method.

Purpose: Early distal muscle weakness and myotonia are typical clinical presentations in type I myotonic dystrophy (DM1). We present a DM1 case with unusual predominant proximal weakness without action myotonia.

Case report: The chief complaint of this 48-year-old female was difficulty in raising her arms and frequent falling in recent years. On neurological examination, proximal muscle weakness was more pronounced than the distal muscle groups, in addition to facial involvement. Although she did not experience any action myotonia throughout her life, hand and tongue myotonia were readily inducible by percussion during neurological examination. The diagnosis of DM1 was later supported by electromyography and neuropathological studies, and confirmed by molecular testing. The pathological findings in this patient and the characteristic features in typical DM1 patients were briefly reviewed.

Conclusion: The unusual presentation of this DM1 patient suggests the importance of comprehensive neurological examination including percussion of thenar and tongue muscles, even in a patient with atypical distribution of muscle weakness and without a clear personal and family history of myotonia. In addition to molecular testing, muscle biopsy remains supportive in making the diagnosis.

Purpose: Anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis is a rare but treatable autoimmune disorder, characterized by gastrointestinal symptoms, cognitive dysfunction, and central nervous system hyperexcitability.

Case report: Herein, we report a case of an 80-year-old male patient who presented with unexplained diarrhea, weight loss, rapidly progressive dementia, tremors, and myoclonus. His serum tested positive for anti-DPPX antibodies. He was treated with plasma exchange, oral prednisolone, and azathioprine. All his symptoms improved substantially after treatment.

Conclusion: Early recognition of anti-DPPX encephalitis is important because it can be treated with immunotherapy. To the best of our knowledge, this is the first reported case of anti-DPPX encephalitis in Taiwan.

Upon acute ischemic stroke, rapid recanalization of the occluded cerebral vessel via intravenous thrombolytic therapy (IVT) is crucial to achieve good functional outcome. The time window of IVT with recombinant tissue plasminogen activator (rt-PA) has been extended from post-stroke 3 to 4.5 hours. In patients with cerebral penumbra identified using cerebral perfusion imaging, IVT is still beneficial within 4.5 to 9 hours after onset of stroke. For those without clear stroke onset time, DWI-FLAIR mismatch by brain MRI indicates hyperacute infarct and IVT is indicative. For patients with large cerebral vessel occlusion, endovascular thrombectomy (EVT) alone is likely non-inferior to bridging therapy (IVT followed by EVT) and this issue is still under investigation. Serial studies have provided the evidence of safety and risk of IVT in specific groups of patients, such as elderly, anticoagulant users, and those having cerebral microbleeds or seizure. Tenecteplase has higher fibrin selectivity than rt-PA and large clinical trials have demonstrated its great potential for stroke therapy. Future clinical trials are mandatory for therapeutic optimization of IVT, especially in bridging therapy, specific groups of patients, and new thrombolytic agents. Keywords: Acute Ischemic Stroke, Cerebral Infarction, Recombinant Tissue Plasminogen Activator, Tenecteplase, Thrombolytic Therapy.

A 77-year-old woman with a 1 years history of Multiple Myeloma (MM) presented with headache, fatigue, and bone pain. She underwent whole body multi-detector computed tomographic (MD-CT) to evaluate possible lytic bone lesions. MD-CT showed small, multiple osteolytic lesions, particularly at the skull level (Figure 1, 2). MM is a plasma cell disorder. It is characterized by the monoclonal proliferation of malignant plasma cells (1,2). These cells, among their various characteristics, determine an infiltrate haemopoietic locations (1). Pathogenesis of MM related bone disease is the uncoupling of the bone remodelling process. There is an increased activity of osteoclastogenesis with the suppressed osteoblastic one, resulting in bone loss (1- 3). This process creates lytic lesions without reactive bone formation (2). Bone disease could be from single lytic lesion to multiple lytic lesions affecting any part of skeleton, preferably skull, spine and long bones (3). MD-CT, with dedicated low-dose protocols, is able to provide whole body skeletal volume information with a greater sensitivity than conventional X-ray studies in MM patients (3). Whole body CT with lowdose protocols can detect lesions with less than 5% trabecular bone destruction, and it is the first-line diagnostic imaging procedure for the diagnosis of lytic bone disease in patients affected by MM (4). When skull is involved, its most common MD-CT presentation is by numerous, well-circumscribed and punched-out lytic bone lesions, without reactive bone formation and diffuse osteopenia (1-5), as in the case presented.

Purpose: To evaluate the relationship between the severity of clinical symptoms and cognitive function of patients with Parkinson's disease (PD) and the serum vitamin D level and nutrition status.

Methods: Thirty-three adult PD patient were included in the study (November 2016 to October 2018) and their clinical symptom severity (including the Hoehn and Yahr scale and unified Parkinson's disease rating scale (UPDRS)) and cognitive function (mini-mental state examination) were assessed in two visits (at time of enrollment and one year after the enrollment). In the meanwhile, their renal/liver function, serum level of vitamin D, vitamin B12, Folate and high-sensitive C-reactive protein were also measured for clinical correlation and comparisons.

Results: From the two visits, we found our patients divided into two group, the well-nourished status group and at risk or malnutrition status group. In both visits, we uncovered patients at risk of malnutrition status had worse clinical severity and more impaired memory. As for hypovitaminosis D, the vitamin D level alone made no significant correlation with the clinical severity and cognitive function.

Conclusion: This study revealed that PD patient with at risk of malnutrition status has impaired cognitive function but patients with abnormal serum vitamin D level did not have such influence. But PD patients with abnormal vitamin D level have a higher hs-CRP level which has an influence on the cognitive function of PD patients. Therefore, abnormal serum vitamin D level may have an indirect influence on the cognitive function of PD patients through the influence on the hs-CRP level. This study is limited by the small case-number and short follow-up time. Further large scale study and longer observation period are needed for a better delineation of the relationship between the serum vitamin D level and nutritional status with the clinical condition of the PD patients.

Purpose: Paroxysmal sympathetic hyperactivity (PSH) occurs in around 15-33% patients of traumatic brain injury. Due to presence of non-specific symptoms, it's always difficult to differentiate between paroxysmal sympathetic storm and cytokine storm syndrome and hence can delay specific treatment.

Case report: We report a clinical case of 19-year-old male tested COVID 19 positive with diffuse axonal injury presented with features of paroxysmal sympathetic storm and cytokine storm syndrome. The patient showed the signs clinical improvement when we treated both these conditions.

Conclusion: We suggest that clinicians need to have a high degree of suspicion of paroxysmal sympathetic storm in patients of traumatic brain injury and consider its diagnosis. Also, if patient is COVID 19 positive, early identification of signs of developing cytokine storm with monitoring of biomarkers is important for its timely management.

Background: Recurrent seizure in epileptic children is correlated with future motoric disorders, behavior problems, and intellectual disabilities. Various factors are thought to modulate the risk of recurrent seizure, including micronutrient status such as calcium, 25-dehydroxycholecalciferol (25-(OH)D), and serum iron presented as hemoglobin level.

Aim: To analyze correlation between micronutrient characteristics of epileptic children and recurrence of seizure.

Methods: This cross-sectional retrospective study was conducted in the pediatric clinic of Dr. Soetomo hospital from September to October 2019. Epileptic children with long-term anti-epileptic drugs (AED) for over 6 months and ages ranging 2-18 years were included. Recurrent and non-recurrent group were compared. Age, family history of seizure, and duration of AED administration were noted. Peripheral serum level of hemoglobin, calcium, and 25-(OH)D was measured. The median 25-(OH) D level of both groups were corelated with recurrent seizure by using Spearman test (95% confidence interval).

Results: Thirty children were enrolled. Recurrent seizure was occurred in 19 children. There was significant correlation on hemoglobin and calcium, and 25-(OH)D level with the recurrence of seizure (p less then 0.05). Among all observed micronutrients, 25-(OH)D has the strongest correlation (r = 0.750). There was no significant correlation between recurrent seizure and both family history and AED administration duration.

Conclusion: Micronutrients status is correlated with prevalence of recurrent seizure. Level of 25-(OH)D is strongly correlated, whereas level of hemoglobin, and calcium have weak correlation with recurrent seizure in epileptic children.

Purpose: Paraneoplastic neurological disorders associated with autoantibodies are rare diseases, causing abnormal manifestations in the central or peripheral nervous system separately or simultaneously. Early recognizing the occurrence of paraneoplastic syndrome can lead to prompt and effective management.

Case report: We presented a patient of subacute cerebellar degeneration with cachectic and bed-ridden status, who was proven to have positive SOX1 antibody. A coexisting Lambert-Eaton myasthenic syndrome was also documented by electrophysiological study.

Conclusion: Intensive and regular follow up for an occult malignancy is crucial in patients with SOX1 antibody. Coadministration of therapies for underlying malignancy and LEMS improve the functional disability.