Acta Neurologica Taiwanica[JOURNAL]
Sun
200 papers
Transient Postictal Hyperglycemia as a Diagnostic Clue of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes.
Liao NY, Lin LY, Chen C … +2 more , Liao YC, Lee YC
Acta Neurol Taiwan · 2022 Mar · PMID 35266136
PURPOSE: To propose that transient postictal hyperglycemia as a diagnostic clue of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). CASE REPORT: We reported two non-diabetic patients pr...
PURPOSE: To propose that transient postictal hyperglycemia as a diagnostic clue of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). CASE REPORT: We reported two non-diabetic patients presenting with generalized seizure and transient postictal hyperglycemia. At the acute stage, both patients had hyperglycemia with serum glucose levels more than 400 mg/dl, normal glycated hemoglobin (HbA1C) levels, normal ketone body levels, and absence of infection signs. Within three days of the seizure event, both patients were euglycemic and did not require any diabetes treatment. Brain MRI examination revealed gyriform restricted diffusion at bilateral superior temporal gyrus in one patient, and diffuse cerebral and cerebellar atrophy without restricted diffusion lesions in another patient. Polymerase chain reaction and restriction fragment length polymorphism (RFLP) analysis confirmed that both patients harbored the m.3243A more than G mutation. CONCLUSION: Seizure-induced stress hyperglycemia is uncommon in normal individuals, but such kind of energy crisis may be pronounced in patients with mitochondrial dysfunction. Early diagnosis of mitochondrial diseases-related epilepsy and hyperglycemia is crucial since certain antiepileptic drugs (ex. Valproic acid) and antihyperglycemic agents (ex. Metformin) are contraindicated in patients with mitochondrial diseases. Our findings support that transient postictal hyperglycemia may be a red flag to consider the diagnosis of MELAS.
Basilar Artery Fenestration Versus Hypoglycemia-Induced Vertebrobasilar Insufficiency: A Case Report.
Tee ZJ, Chen WC, Huang P
Acta Neurol Taiwan · 2022 Mar · PMID 35266135
PURPOSE: Vertebrobasilar insufficiency (VBI) is a common transient neurological condition related to posterior circulation hemodynamic insufficiency. However, it is rarely seen as an initial presentation in basilar arter...
PURPOSE: Vertebrobasilar insufficiency (VBI) is a common transient neurological condition related to posterior circulation hemodynamic insufficiency. However, it is rarely seen as an initial presentation in basilar artery (BA) fenestration or hypoglycemia. We present this case to further clarify the association between BA fenestration and hypoglycemia-induced VBI, as well as the difficulty in diagnosis, especially in acute clinical settings. CASE REPORT: Herein, we report a case with BA fenestration, in which the patient suffered from transient episodes of focal neurological deficits, including dysarthria, focal limbs weakness, and ataxia with subsequent total recovery. Apart from hypoglycemia, no other abnormal laboratory surveys were found. This concurrent finding of hypoglycemia with transient focal neurological deficit poses a difficulty in differentiating between hypoglycemia-induced VBI and true acute ischemic stroke in the clinical setting. Subsequent brain imaging studies revealed no evidence of acute infarction and no evidence of atherosclerosis changes in vessels but BA fenestration was observed. We prescribed antiplatelets for the prevention of future strokes. However, currently, no consensus exists regarding the prevention of cerebral ischemia with BA fenestration. CONCLUSION: BA fenestration-induced VBI and hypoglycemia-induced VBI are rarely reported and their mechanisms of action remain uncertain and controversial. However, BA fenestration-induced VBI may pose a risk for future cerebral ischemic events and warrants further investigations.
Primary Familial Brain Calcification Caused by a Novel Compound Heterozygous Mutation in the MYORG Gene.
Tsai MC, Lee JT, Sung YF … +1 more , Yang FC
Acta Neurol Taiwan · 2022 Mar · PMID 35266134
BACKGROUND PURPOSE: To demonstrate a novel compound heterozygous mutation in MYORG-related recessive primary familial brain calcification. CASE REPORT: We report a case of primary familial brain calcification with newly-...
BACKGROUND PURPOSE: To demonstrate a novel compound heterozygous mutation in MYORG-related recessive primary familial brain calcification. CASE REPORT: We report a case of primary familial brain calcification with newly-discovered compound heterozygous mutation in the MYORG gene presenting with progressive parkinsonism, cerebellar signs, and typical diffuse brain calcifications. CONCLUSION: Clinicians should consider MYORG testing in patients who have primary brain calcifications with either a negative or recessive family history.
Effect of Chronic Valproic Acid Use on Anterior Cerebral Blood Flow of Children with Idiopathic Generalized Epilepsy.
Ozyurek H, Bayrak I, Yayici Koken O … +3 more , Ince H, Tasdemir HA, Aydin OF
Acta Neurol Taiwan · 2022 Mar · PMID 35266133
OBJECTIVE: Cerebral blood flow has been blamed as a factor in the negative effect of antiepileptic drugs on neurocognition. This study aimed to investigate whether valproic acid (VPA), used for the treatment of idiopathi...
OBJECTIVE: Cerebral blood flow has been blamed as a factor in the negative effect of antiepileptic drugs on neurocognition. This study aimed to investigate whether valproic acid (VPA), used for the treatment of idiopathic generalized epilepsy (IGE), causes a change in cerebral blood flow in children. METHODS: Included in this study were 33 children who were receiving VPA for IGE and 34 age-matched controls. Doppler and spectral measurements in common carotid artery (CCA), left and right internal CA (ICA) and external CA (ECA), anterior cerebral artery (ACA) and middle cerebral artery (MCA) were performed and the maximum velocity (VM), end-diastolic velocity (EDV), resistive index (RI), pulsatility index (PI) and flow rate (FR) were calculated. RESULTS: The mean age of drug and control groups were 9.33 plus or minus 2.11, and 9.74 plus or minus 2 years, respectively. Follow-up of patients was 17.7 plus or minus 3.2 months. The period of VPA treatment was 17.4 plus or minus 3.4 months. No statistically significant differences were found between control and VPA group for the VM, EDV, RI, PI, and FR values obtained from the bilateral ICA, ACA, and MCA. CONCLUSIONS: The results showed that VPA in therapeutic doses did not affect anterior cerebral blood flow. However according to result, it is still difficult to conclude that neurocognitive deterioration is not observed in patients receiving VPA.
Accuracy of Cerebrospinal Fluid C-Reactive Protein and Multiplex Polymerase Chain Reaction and Serum Procalcitonin in Diagnosis of Bacterial and Viral Meningitis in Children.
Ahmed MA, Askar GA, Farghaly HS … +4 more , Ahmed AO, Kamal DT, Ahmed SS, Mohamad IL
Acta Neurol Taiwan · 2022 Mar · PMID 35266132
BACKGROUND: Meningitis is one of the most dangerous infection affecting children. The need for rapid and accurate diagnosis is mandatory for improving the outcome. AIM OF THE WORK: to evaluate the role of multiplex polym...
BACKGROUND: Meningitis is one of the most dangerous infection affecting children. The need for rapid and accurate diagnosis is mandatory for improving the outcome. AIM OF THE WORK: to evaluate the role of multiplex polymerase chain reaction (PCR), cerebrospinal fluid (CSF)-C-reactive protein (CRP) and serum procalcitonin (PCT) in diagnosis of meningitis and to detect its accuracy. PATIENTS AND METHODS: A cross-sectional study was carried out in University Children hospital, Faculty of Medicine, between November 2019 and September 2020. The study was approved by the Ethics Review Board of Faculty of Medicine, Assiut University, and informed written consent was obtained. The committee's reference number is 17200161. CLINICALTRIALS: gov ID: NCT03387969. 48 Children aged 2 to 18 years with meningitis were included. Detailed history and examination. Blood glucose level at time of admission prior to lumbar puncture, serum CRP level, serum PCT, CSF-CRP level and Multiplex PCR were evaluated. FUNDING: The study was supported by Grant Office of Faculty of Medicine, Assiut University with grant NO. 2018-01-04-006-R2. RESULTS: The mean age of children was 3.27 plus or minus 1.27 years. 35 (72.9%) cases were bacterial meningitis, while 13 (27.1%) cases were viral meningitis. Patients with bacterial meningitis had significantly higher serum CRP, serum PCT and higher CSF-CRP and significantly lower CSF/blood glucose compared to viral meningitis. Multiplex PCR had 94% sensitivity and 100% specificity for diagnosis of bacterial and viral meningitis. CONCLUSION: CSF-CRP, CSF/blood glucose, PCT and Multiplex-PCR may help in diagnosis and differentiation of bacterial and viral meningitis.
Biomarkers with Plasma Amyloid β and Tau Protein Assayed by Immunomagnetic Reduction in Patients with Amnestic Mild Cognitive Impairment and Alzheimer's Disease.
Lee PJ, Tsai CL, Liang CS … +5 more , Peng GS, Lee JT, Tsai CK, Lin YK, Yang FC
Acta Neurol Taiwan · 2022 Jun · PMID 35040113
This review addresses recent developments in the analyses of plasma amyloid beta (Aβ) and total tau (t-tau) protein levels as biomarkers for discriminating amnestic mild cognitive impairment (aMCI) from Alzheimer disease...
This review addresses recent developments in the analyses of plasma amyloid beta (Aβ) and total tau (t-tau) protein levels as biomarkers for discriminating amnestic mild cognitive impairment (aMCI) from Alzheimer disease (AD), using immunomagnetic reduction (IMR). Recent studies have focused on the differential diagnosis of normal controls (NCs) with aMCI or AD. Results of 15 clinical studies have demonstrated decrease in plasma Aβ1-40 and increase in plasma Aβ1-42 and t-tau levels in patients with aMCI and AD. For a given biomarker, effect size is determined by comparing the mean ratios of biomarker levels between two diagnostic groups. Effect sizes are less than 1 for Aβ1-40 (0.606-1.032) but >1 for Aβ1-42 (1.018-2.167) and t-tau (1.030-4.147) in aMCI and AD compared with NCs. The effect size of the plasma tau significantly increases the most as aMCI progresses to AD. Studies into the application of IMR to determine plasma Aβ and tau levels as biomarkers for aMCI or AD have recently progressed. Future investigations should validate recently published results, preferably in patients with pathologically confirmed AD. In addition, effort should be directed toward standardizing the design of such studies and data analysis. Keywords: amyloid beta, plasma tau, Alzheimer disease, biomarker, mild cognitive impairment.
Postvaccinal Encephalopathy Presenting with Amnesia and Seizure After ChAdOx1 nCov-19 Vaccination: A Case Report.
Huang YJ, Huang CS
Acta Neurol Taiwan · 2022 Sep · PMID 35040112
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Thymoma Removal Improved Cognitive Function in a Patient with Alzheimer disease: A Case Report.
Lee LH, Yip PC, Fan YM … +1 more , Liu YC
Acta Neurol Taiwan · 2022 Sep · PMID 35040111
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An unusual case of recurrent subdural hematoma.
Jha M, Narayanan B, Sharma A … +1 more , Bhatia P
Acta Neurol Taiwan · 2022 Sep · PMID 35040110
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Japanese encephalitis with unilateral thalamic lesion: Clinical Pictures.
Huang WC, Lin CM
Acta Neurol Taiwan · 2022 Sep · PMID 35040109
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The First Guillain-Barr? Syndrome After SARS-CoV-2 Vaccination in Taiwan.
Su SC, Lyu RK, Chang CW … +1 more , Tseng WJ
Acta Neurol Taiwan · 2022 Jan · PMID 34988954
PURPOSE: Guillain-Barre syndrome (GBS) is an immune-mediated disease of the peripheral nerves and could be fatal and has severe neurologic complications. This study herein reports the clinical course of the first patient...
PURPOSE: Guillain-Barre syndrome (GBS) is an immune-mediated disease of the peripheral nerves and could be fatal and has severe neurologic complications. This study herein reports the clinical course of the first patient of GBS after SARS-CoV-2 Oxford/AstraZeneca vaccination in Taiwan. CASE REPORT: A 38-year-old woman who presented with progressive numbness and weakness of both upper and lower limbs over 1 week. Ascending patterns was noted, and bilateral leg were more severe with diffused absence of deep tendon reflex. Clinical examination and investigation findings confirmed with the diagnosis of GBS. Deterioration of muscle power and respiratory failure had developed during the hospitalization. She had no common GBS predisposing history, but she had received her first SARS-CoV-2 Oxford/AstraZeneca vaccination intramuscularly 10 days prior to her symptoms. Clinical symptoms had much improved after double filtration plasmapheresis. CONCLUSION: Our case is the first case of GBS developed after AstraZeneca vaccine injection in Taiwan, presenting with atypical manifestation of early facial and bulbar involvement. The vaccination associated GBS should be closely monitored as other safety profile, since it may result in respiratory failure and severe neurologic complications. Keyword: Guillain-Barre Syndrome, SARS-CoV-2 Vaccination.
Cefepime Induced Neurotoxicity Mimicking Clinical Presentation of Left Middle Cerebral Artery Infarction: A Case Report and Review of Literature.
Su CY, Lin WH
Acta Neurol Taiwan · 2022 Jan · PMID 34988953
PURPOSE: Cefepime is a widely used antibiotic which was known to have neurotoxicity resulted from its ability to cross the blood-brain barrier and a wide variety of symptoms had been documented. Here we reported a case o...
PURPOSE: Cefepime is a widely used antibiotic which was known to have neurotoxicity resulted from its ability to cross the blood-brain barrier and a wide variety of symptoms had been documented. Here we reported a case of Cefepime induced neurotoxicity with rare presentation. The aim of this study was to improve the knowledge of this condition. CASE REPORT: A 89-year-old female with a history of ESRD (end stage renal disease) and superimposed acute cholecystitis was treated with Cefepime. She developed the symptoms of global aphasia, right hemiplegia, leftward eye deviation and abnormal plantar reflex at right foot, which resembled acute ischemic stroke at left MCA (middle cerebral artery), on the fourth day of Cefepime treatment. There was no evidence of acute infarction in MRI (magnetic resonance imaging) of brain and EEG (electroencephalography) revealed NCSE (nonconvulsive status epilepticus). NCSE was suspected to be attributed to Cefepime-induced neurotoxicity. The patient's main risk factors were decreased renal clearance and incorrect dosing. Conslusion: Cefepime-induced neurotoxicity should be suspected in patients who developed neurologic symptoms after the administration of Cefepime. Emergent image study for excluding more commonly seen or critical etiologies and further evaluation with EEG were necessary. For those patients who have risk factors for Cefepime neurotoxicity, such as ESRD, TDM (therapeutic drug monitoring) may be useful in providing close monitoring and preventing adverse effects associated with Cefepime treatment. Keyword: Cefepime, acute ischemic stroke, aphaia, nonconvulsive status epilepticus.
Neuroendocrine Carcinoma of the Endometrium with Ectopic Secretion of Parathyroid Hormone Presenting as Hypercalcemia- Related Posterior Reversible Encephalopathy syndrome: A Case Report.
Lin W, Lin YH, Gao HW … +4 more , Chen IF, Ko CA, Tsai CK, Lin YK
Acta Neurol Taiwan · 2022 Jan · PMID 34988952
PURPOSE: Posterior reversible encephalopathy syndrome (PRES) displayed various acute neurological symptoms. PRES is a rare presentation of hypercalcemia. Here we present a case with ectopic secretion of parathyroid hormo...
PURPOSE: Posterior reversible encephalopathy syndrome (PRES) displayed various acute neurological symptoms. PRES is a rare presentation of hypercalcemia. Here we present a case with ectopic secretion of parathyroid hormone from neuroendocrine carcinoma of the endometrium presenting as hypercalcemia-related PRES. CASE REPORT: A 67-year-old woman presented with acute generalized tonic-clonic seizure followed by post-ictal confusion and neuropsychiatric behaviors. The diagnosis is PRES. Investigations showed uterine cervical region with multiple liver metastasis complicated with hypercalcemia, elevated intact parathyroid hormone. Further pathology concluded as a poorly differentiated adenocarcinoma of the endometrium with neuroendocrine differentiation and immunoreactive for PTH. The patient's neurologic manifestations had resolved. Serum free calcium level and intact-PTH had declined after first course of definitive chemoradiation. CONCLUSION: Immunostaining of the tumor tissue can be used to estimate the ectopic PTH production within the tumor cells. Early detection and appropriate clinical treatment hold the potential to improve the prognosis of refractory hypercalcemia and hypercalcemia related PRES. Keyword: Posterior reversible encephalopathy syndrome; hypercalcemia; intact-parathyroid hormone; parathyroid hormone-related peptide; neuroendocrine carcinoma of endometrium.
Adaptation and Validation of the Hamburg Quality of Life Questionnaire in Multiple Sclerosis (HAQUAMS) for Use in Iranian Patients.
Rezaei S, Saberi A, Hatamian H … +3 more , Ghayeghran A, Khaksari Z, Mollahosein F
Acta Neurol Taiwan · 2022 Jan · PMID 34988951
PURPOSE: Quality of life (QoL) is considered as an important criterion for therapeutic effectiveness. Therefore, the present study aimed to validate the Persian version of the Hamburg Quality of Life Questionnaire in Mul...
PURPOSE: Quality of life (QoL) is considered as an important criterion for therapeutic effectiveness. Therefore, the present study aimed to validate the Persian version of the Hamburg Quality of Life Questionnaire in Multiple Sclerosis (HAQUAMS) for use in Iranian people with MS. METHODS: In a cross-sectional study, 158 people with MS were selected through the census sampling method. The construct validity of the Persian version of HAQUAMS was first evaluated by a confirmatory factor analysis (CFA) in AMOS-22 software, and then the internal consistency reliability and the item-total score correlations were calculated for each subscale by the SPSS-22. RESULTS: The CFA and output results indicated that the HAQUAMS with a five-factor structure among the Iranian MS patients had a good construct validity if an item was eliminated and a number of covariance errors between items were released (RMSEA is euqal to 0.069). The internal consistency of HAQUAMS subscales was acceptable to excellent (alpha is euqal to 0.81 to 0.91). The analysis of item-total score correlation for determining the construct validity of HAQUAMS indicated that all items of the questionnaire had a moderate to strong positive correlation with their subscales (P less than 0.0001, r is euqal to 0.41 to 0.89). The correlation of total scores of HAQUAMS and the Beck Depression Inventory-short form (BDI-13) was equal to 0.74 (P less than 0.0001), indicating good concurrent criterion validity. CONCLUSION: The Persian version of the HAQUAMS with a five-factor construct had acceptable validity and reliability and could be used for measurement of the health related QoL in Iranian people with MS.
Study of the Demographic and Clinical Profile in a Neurocutaneous Rare Disease: A Cross-Sectional Study.
Jouybari L, Foji S, Sanagoo A … +2 more , Oladenabidozin M, Yazdani A
Acta Neurol Taiwan · 2022 Jan · PMID 34988950
OBJECTIVE: Neurofibromatosis is one of the most common dominantly inherited genetic disorders. This study aimed to study the demographic and clinical profile of neurofibromatosis patients. METHODS: This study is cross-se...
OBJECTIVE: Neurofibromatosis is one of the most common dominantly inherited genetic disorders. This study aimed to study the demographic and clinical profile of neurofibromatosis patients. METHODS: This study is cross-sectional conducted in 2020 on the population of patients with neurofibromatosis. Patients who are members of the Neurofibromatosis Association answered the online demographic and clinical information questionnaire. RESULTS: 446 patients with neurofibromatosis participated in this study with a mean age of 33.39 plus or minus 12.87 years. 297 patients (66.6%) were women and 378 (84.8%) patients had type 1 neurofibromatosis. The disease visibility was reported to be moderate in 254 patients (54.9%) and the severity of the disease was mild in 238 (53.4%) patients. The type of neurofibromatosis was not significantly related to gender, age groups, parental education, and ethnicity. The relationship between severity and age (p is equal to less than 0.001) and gender (p is equal to 0.042) was significant and the relationship between visibility and age (p is equal to less than 0.001) was significant but despite the fact that the disease was more visible in men than women, it was not significantly related to gender. CONCLUSIONS: The study results showed that the most common complication in the study population was Cafe au lait spot. In addition, visibility and severity of the disease were mild and moderate, respectively. Keyword: Neurofibromatosis, Demographic information, Clinical Information.
The Effect of Month of Birth on Multiple Sclerosis: A Sase-Control Study in Northeast Iran.
Sarmadi M, Nezhad MR, Hasanluyi FA … +3 more , Najafi F, Tamaddon A, Rahimi S
Acta Neurol Taiwan · 2022 Jan · PMID 34988949
BACKGROUND: Multiple sclerosis (MS) is an autoimmune and multi-factorial (e.g. environmental, genetic) disease. We conducted a case-control study of month and season of birth (MOB and SOB) and multiple sclerosis (MS) ris...
BACKGROUND: Multiple sclerosis (MS) is an autoimmune and multi-factorial (e.g. environmental, genetic) disease. We conducted a case-control study of month and season of birth (MOB and SOB) and multiple sclerosis (MS) risk in the east of Iran. METHODS: The MS patients registered in Mashhad and Torbat Heydariyeh MS Society until 20 March 2018 was compared with the MOB and SOB in the healthy population during 1988 to 2018. Case group was matched for age, sex and place of residence with the control group. Differences in the distributions of MOB and SOB between the patients and the control groups were assessed using the chi-square test. RESULTS: There were 2,160 MS patients in case group and 2,245 in control group. There was a significant relationship between MOB ans SOB with the risk of MS (P less than 0.05). Analysis showed a significant (p less than 0.01) peak in the MOB during Mar-Apr (OR is equal to 1.60), May-Jun (OR is equal to 1.30) and Aug-Sep (OR is equal to 2.42). CONCLUSION: The findings show a relationship between MOB and SOB as risk factor for MS in Northeast Iran. Further studies are needed to confirm this result. Keyword: Multiple sclerosis, Month of Birth, seasonality, case-control, Iran.
Exosomes-Potential for Blood-Based Marker in Alzheimer's Disease.
Li CC, Hsu WF, Wo AM
Acta Neurol Taiwan · 2022 Jan · PMID 34988948
Exosomes are believed to be secreted from multivesicular endosomes and containing proteins and nucleic acids, including mRNA and microRNAs, which have been implicated to play a role in neurodegenerative diseases. Neuron-...
Exosomes are believed to be secreted from multivesicular endosomes and containing proteins and nucleic acids, including mRNA and microRNAs, which have been implicated to play a role in neurodegenerative diseases. Neuron-derived exosomes at the circulation provide a unique potential as biomarkers towards assessment of Alzheimer's disease (AD), even at the pre-clinical stage. This review briefly discusses their biogenesis and transport, exosomal protein verses soluble protein, evidence for their role in AD, isolation of exosomes, and challenges and future directions to realize reliable blood-based biomarkers to meet phenomenal unmet clinical and pre-clinical need of AD.
Early Rehabilitation after Acute Stroke:The Golden Recovery Period.
Liu Y, Yin JH, Lee JT … +2 more , Peng GS, Yang FC
Acta Neurol Taiwan · 2023 Mar · PMID 34918304
Stroke is a leading cause of disability worldwide. Neuroplasticity, a condition wherein the brain's dynamic response to injury is heightened and rehabilitation might be effective, is observed shortly after acute stroke....
Stroke is a leading cause of disability worldwide. Neuroplasticity, a condition wherein the brain's dynamic response to injury is heightened and rehabilitation might be effective, is observed shortly after acute stroke. However, although several trials have demonstrated that initiating treatment within 24 hours after stroke is potentially harmful, some have shown that early rehabilitation of patients is beneficial. Administration of constraint-induced movement therapy within two weeks after stroke appears to be beneficial for the upper extremities. In addition, intensive early post-stroke therapy may be beneficial for patients with severe aphasia. Novel approaches to early treatment of post-stroke dysphagia appear promising; however, the high rate of spontaneous improvement makes it difficult to gauge their benefits. Overall, although increasing evidence indicates that initiating rehabilitative strategies within two weeks after stroke is beneficial for some deficits, the optimal time for initiating post-stroke rehabilitation remains undetermined. Keywords: stroke, early rehabilitation, neuroplasticity, early mobilization.
Multidisciplinary approach to Fibromyalgia: What are we learning from updated evidence-based medicine?
Yin JH, Peng GS, Ro LS
Acta Neurol Taiwan · 2022 Dec · PMID 34918303
Abstract loading — click title to view on PubMed.
Treatable Rapid Progressive Dementia: A First Case Report of Anti-dipeptidyl-peptidase-like Protein 6 Encephalitis in Taiwan.
Tsai MC, Shih HK, Lin HH … +2 more , Tsai MY, Sung YF
Acta Neurol Taiwan · 2021 Sep · PMID 34841508
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 hyperexcita...
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.
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