Acta Neurologica Taiwanica[JOURNAL]
Sun
200 papers
A Myotonic Dystrophy Type I patient with Predominant Proximal Muscle Weakness without Action Myotonia- A Case Report and Review of Pathology.
Wu CI, Chien YY, Lee KY
Acta Neurol Taiwan · 2021 Sep · PMID 34841507
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 REP...
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.
A 50 Year-Old Woman with A Delayed Diagnosis of Neuromyelitis Optica Spectrum Disorder: The Clinical Course and Serial Neuroimaging Findings.
Huang YJ, Chang WN
Acta Neurol Taiwan · 2021 Sep · PMID 34841506
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...
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.
Assessment of small intestinal bacterial overgrowth in Alzheimer's disease.
Sim J, Wang YT, Mamun K … +4 more , Tay SY, Doshi K, Hameed S, Ting SK
Acta Neurol Taiwan · 2021 Sep · PMID 34841505
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...
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.
Spectrum of Movement Disorders in two Movement Disorders Centers in the Philippines.
Yu JRT, Jamora RDG, Silverio EL … +4 more , Bautista JMP, Luspian KJL, Tiongson RM, Ng AR
Acta Neurol Taiwan · 2021 Sep · PMID 34841504
OBJECTIVE: Presently, there are no epidemiologic data on the prevalence of movement disorders in the Philippines. We aim to describe the most common phenomenologies and movement disorders in two specialty centers in Metr...
OBJECTIVE: Presently, there are no epidemiologic data on the prevalence of movement disorders in the Philippines. We aim to describe the most common phenomenologies and movement disorders in two specialty centers in Metro Manila dedicated to movement disorders. METHODS: We investigated the clinical spectrum and etiologies of movement disorders referred to our centers from January 2007-December 2019 using a standardized collection form. RESULTS: A total of 1438 patients presenting with complaints relating to movement disorders were evaluated between 2007 to 2019. There were 770 (53.5%) men. The mean age was 57.1 ± 17.9 years. The most common movement disorders were parkinsonism (n=677, 47.1%), myoclonus (n=212, 14.7%) and tremor (n=208, 14.5%). The least common was restless legs syndrome (n=4, 0.3%). There were 78 (37.7% of total dystonia cases) X-linked dystonia-parkinsonism patients referred to our clinic. Majority of the botulinum toxin injections were for hemifacial spasms (n=206). A small number of patients (n=41) were also seen at the center for deep brain stimulation programming. CONCLUSION: The most common movement disorders managed were parkinsonism, myoclonus and tremor. The most common diagnoses were Parkinson's disease, hemifacial spasm and essential tremor. This study highlights the spectrum of movement disorders encountered in two specialty clinics in two Philippine tertiary hospitals. Given these varied cases, there is also a need for more movement specialists and centers dedicated to movement disorders to manage these cases.
Updates on the Genetics of Parkinson's Disease: Clinical Implications and Future Treatment.
Chu YT, Tai CH, Lin CH … +1 more , Wu RM
Acta Neurol Taiwan · 2021 Sep · PMID 34841503
Parkinson' disease (PD) is a common neurodegenerative disease with the pathological hallmark of alpha-synuclein aggregation within dopaminergic neurons. The etiology of PD comes from a complex interplay between genetic a...
Parkinson' disease (PD) is a common neurodegenerative disease with the pathological hallmark of alpha-synuclein aggregation within dopaminergic neurons. The etiology of PD comes from a complex interplay between genetic and environmental factors. Though most cases of PD are sporadic; a family history of PD is found in approximately 15% of patients. Pathogenic mutations are found in 5% to 10% of individuals with either familial or sporadic PD. In recent decades, because of the advent of next generation sequencing, more than 25 genes have been identified as causative genes in PD. These findings allow better understanding of the pathogenesis of PD, including aberrant alpha-synuclein homeostasis, defective mitochondrial functions, and impairment of the ubiquitin-proteasome and autophagy-lysosome pathways. Among the PD-causative genes, LRRK2 mutation is the most frequent mutation in autosomal dominant PD and Parkin mutation is prevalent in patients with autosomal recessive or early onset PD. Several genetic epidemiology studies in Asians have revealed a distinctive mutation spectrum from Western populations, reinforcing the importance of ethnic differences in PD. Proper genetic testing is recommended for patients with early onset, a strong family history, or associated red flag clinical features. Considering that clinical trials of disease-modifying therapy targeting patients with specific mutations are ongoing and we are in the era of precision medicine, this review highlights recent updates of genetic findings in patients with PD, focusing on Asian populations and practical recommendations for genetic testing. Keywords: Parkinson's disease, Genetics.
A Study of Seven Patients with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) in Eastern Taiwan: A Case Series with Literature Review.
Thu PW, Lo RY
Acta Neurol Taiwan · 2021 Dec · PMID 34841502
PURPOSE: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is the most common cause of heritable vascular dementia. Recognizing the disease before the full-blown clinica...
PURPOSE: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is the most common cause of heritable vascular dementia. Recognizing the disease before the full-blown clinical features is challenging, so our case series high light clinical characteristics, screening tools and diagnostic process of the patients with CADASIL. CASE REPORT: Our case series reports neurocognitive features, neuroimaging, and exemplary pedigrees of seven patients with genetically confirmed CADASIL, in which six patients presented with dementia and the other one presented with migraine. CONCLUSION: Our report is the single-center experience of our hospital in eastern Taiwan, where access to medical care and genetic test is relatively limited compared to other parts of Taiwan. We had also compared the utility of Davous' CADASIL criteria and the CADASIL scale, and both can be used as sensitive screening tools before genetic tests, especially in the area with limited medical access.
Cerebral Venous Thrombosis Mimicking Acute Ischemic Stroke in the Emergency Assessment of Thrombolysis Eligibility: Learning from a Misdiagnosed Case.
Lin PY, Chen YC, Sun YT
Acta Neurol Taiwan · 2021 Dec · PMID 34841501
PURPOSE: Cerebral venous thrombosis (CVT) occasionally presents with acute focal neurologic signs, mimicking arterial stroke syndrome. Diagnosing CVT in the setting of thrombolysis eligibility evaluation is challenging....
PURPOSE: Cerebral venous thrombosis (CVT) occasionally presents with acute focal neurologic signs, mimicking arterial stroke syndrome. Diagnosing CVT in the setting of thrombolysis eligibility evaluation is challenging. We reported this case to discuss the promptly recognizing CVT in the setting of thrombolysis eligibility evaluation, and review the literature of thrombolytic therapy in CVT patients. CASE REPORT: A 57-year-old man presented with acute-onset right upper extremity monoparesis, right facial palsy, and aphasia. He underwent emergent thrombolysis with recombinant tissue plasminogen activator according to American Stroke Association guidelines. Subsequently, CVT was identified on multiphase computed tomography (CT) angiography. His symptoms initially improved but subsequently deteriorated because of intracranial hemorrhage. Cryoprecipitate and tranexamic acid were immediately administered. Anticoagulation was started 24 hours after the onset of hemorrhage. His modified Rankin Scale score was 4 at 120 days after the hemorrhage. CONCLUSION: Patients with CVT have a higher risk of thrombolysis-related intracranial hemorrhage than other stroke mimics. A greater focus on noncontrast brain CT and the venous phase of CT angiography help identifying this stroke mimic before thrombolysis.
Symptomatic Migraine with Prolonged Visual Aura and Unruptured Occipital Arteriovenous Malformation.
Liu WT, Lin CJ, Yeh PS
Acta Neurol Taiwan · 2021 Dec · PMID 34841500
A 35 year-old woman had a two-year history of recurrent headache with clinical presentations of visual aura in her left visual field followed by right-sided throbbing headache. The patient suffered from a similar attack...
A 35 year-old woman had a two-year history of recurrent headache with clinical presentations of visual aura in her left visual field followed by right-sided throbbing headache. The patient suffered from a similar attack but her visual aura-like symptoms persisted for over 48 hours. The concurrent electroencephalogram demonstrated focal non-epileptiform rhythmic slow waves in the right occipital region. The magnetic resonance images showed prominent parenchymal edema in the right occipital area. The cerebral angiographic study proved a small cerebral arteriovenous malformation. This illustrated case showed that cerebral arteriovenous malformation produces headaches mimicking migraine with visual aura. The acute vascular flow change and the parenchymal edema trigger a prolonged visual aura with coinstantaneous evidence of cortical depression shown on the electroencephalogram. Keywords: Symptomatic migraine; Prolonged visual aura; Unruptured arteriovenous malformation.
The Clinical Characteristics and Therapeutic Outcomes of Escherichia Coli Meningitis in Adults.
Hsiao WC, Lee JJ, Chien CC … +3 more , Lu CH, Chang WN, Lien CY
Acta Neurol Taiwan · 2021 Dec · PMID 34841499
BACKGROUND: To examine the clinical characteristics and therapeutic outcome of Escherichia (E.) coli adult bacterial meningitis (ABM). METHODS: The demographic data, clinical and laboratory features and therapeutic outco...
BACKGROUND: To examine the clinical characteristics and therapeutic outcome of Escherichia (E.) coli adult bacterial meningitis (ABM). METHODS: The demographic data, clinical and laboratory features and therapeutic outcome of 25 E. coli ABM patients were examined retrospectively. The clinical features of the reported E. coli ABM cases were also included for analysis. RESULTS: The 25 E. coli ABM patients included 12 women and 13 men, aged 33-78 years (mean= 59.9). Of these 25 patients, 13 had a postneurosurgical state as the underlying condition. As to the underlying medical conditions, diabetes mellitus was the most common, found in 9 of the 25 cases. Of the clinical manifestation, severe neurologic manifestations including altered consciousness (19), hydrocephalus (10), seizure (7) acute/subacute cerebral infarct (5), brain abscess (2), subdural empyema (1) and spinal abscess (1) were found, and the other clinical features included fever (21), septic shock (8), bacteremia (6) and hyponatremia (3). With treatment, the mortality rate was more than 44.0% and the presence of septic shock was a significant prognostic factor. With literature review, 29 community-acquired and 12 postneurosurgical E. coli ABM cases were enrolled, and severe neurologic manifestation and high mortality rate were also found. CONCLUSIONS: This preliminary overview of E. coli ABM revealed the underlying conditions, severe neurologic manifestation and high mortality rate. Further large-scale, prospective study is needed for a better delineation of this specific infectious syndrome of adult E. coli meningitis.
Acute and chronic bilateral internal carotid artery occlusion.
Chen TY, Chang WL, Chen PY … +2 more , Hsiao CL, Lin SK
Acta Neurol Taiwan · 2021 Dec · PMID 34841498
PURPOSE: Occlusion of both internal carotid arteries (ICAs) is rare. Clinical manifestations of stroke vary widely. We conducted a retrospective review to compare acute and chronic bilateral ICA occlusion. METHODS: We re...
PURPOSE: Occlusion of both internal carotid arteries (ICAs) is rare. Clinical manifestations of stroke vary widely. We conducted a retrospective review to compare acute and chronic bilateral ICA occlusion. METHODS: We retrospectively reviewed records of inpatients with acute ischemic stroke and carotid duplex sonography (CDS) during the period from February 2006 to February 2021. RESULTS: Bilateral ICA occlusion and acute bilateral ICA occlusion accounted for 0.3% and less than 0.1% of all ischemic stroke cases, respectively. All five patients with acute bilateral ICA occlusion presented with consciousness disturbance. Three patients died within 1 week, and two patients had a vegetative outcome. Pituitary apoplexy with bilateral ICA occlusion was observed in one patient. Forward bilateral ophthalmic arterial flow (OAF) was detected in all three patients who received CDS. Among 13 patients with chronic bilateral ICA occlusion, five and six had modified Rankin Scale (mRS) scores upon discharge of more than 5 and less than 2, respectively; two patients did not have a stroke. Of the 13 patients, 11 had reversed bilateral OAF. Patients with acute bilateral ICA occlusion had a higher rate of initial consciousness disturbance, Glasgow Coma Scale score of less than 9, National Institute of Health Stroke Scale score of more than 20, and mRS score of more than 5. than that of patients with chronic bilateral ICA occlusion. CONCLUSION: Patients with acute bilateral ICA occlusion had higher initial stoke severity, poorer collateral circulation, and worse clinical outcomes than did those with chronic bilateral ICA occlusion. Physicians must pay attention to rare causes of acute bilateral ICA occlusion, including pituitary apoplexy.
Management of carotid artery stenosis.
Lee TH
Acta Neurol Taiwan · 2021 Dec · PMID 34841497
The incidence of both ischemic and hemorrhagic stroke is more common in Asians compared to Caucasian (1). It is found there is a decreased incidence of ischemic stroke in Northern America but increased incidence in Afric...
The incidence of both ischemic and hemorrhagic stroke is more common in Asians compared to Caucasian (1). It is found there is a decreased incidence of ischemic stroke in Northern America but increased incidence in Africa, Mongolia and southeast Asia. In the epidemiological study of carotid artery stenosis (CAS), Framingham study showed the prevalence of significant extracranial CAS was 7% in women and 9% in men (2). Among all strokes in Caucasian population, 20 to 30% were due to extracranial CAS and 5 to 10% due to intracranial atherosclerosis (3, 4). Northern Manhattan stroke study also found intracranial atherosclerosis could be seen in 6 to 10% of ischemic strokes in white patients, but up to 29% among African Americans and Hispanics (5). Intracranial artery stenosis was more frequently found in Chinese population than extracranial artery stenosis with the range of 3.7% - 70.4% of intracranial CAS and 1.5%-49% of extracranial CAS (6). Intracranial artery stenosis is more common in Asian, Hispanic, and African-American populations. In hospitalized patients with symptomatic intracranial stenosis, it was only 1% in non-Hispanic whites, while 50% in Asian populations. Population-based studies revealed the prevalence of symptomatic intracranial disease was 1 in 100,000 for whites to 15 in 100,000 for African Americans, but 7% of the population aged more than 40 years for Chinese (7). Concurrent atherosclerosis of extracranial and intracranial arteries was also common in Asians. It was reported 10% to 48% in patients with symptomatic cerebrovascular disease, and 21% of stroke patients had concurrent stenoses in Hong Kong, 33% in China, 18% in Taiwan, and 48% of patients with more than 30% extracranial carotid stenosis had concurrent intracranial stenosis in South Korea (8). The study of concomitant atherosclerotic arterial diseases showed in patients with more than 50% significant CAS, the most frequent artery was coronary artery disease which was found in 68% of patients, while renal artery stenosis and limb artery stenosis were found in 20% and 21% of patients, respectively (9). Carotid artery stenosis of 70% or greater was detected in 37.7% patients with cerebrovascular disease, 24.5% patients with peripheral arterial disease, and 11.1% patients with coronary artery disease (10). Significant extracranial carotid and vertebral artery disease (ECCVD) identified by duplex ultrasonography is not uncommon in Chinese patients with coronary artery disease (CAD), and 22.9% ECCVD was seen in patients with CAD, comparable with that reported in white populations (11). In our Stroke Registry In Chang-Gung Healthcare System (SRICHS) from 2008 to 2011 (Fig. 1), we found large artery atherosclerosis (LAA) occupied 19.5% of total ischemic stroke patients and 3.1% had concurrent atrial fibrillation (12).
Tolosa-Hunt syndrome as initial presentation of Systemic Lupus Erythematosus.
Lee KP, Sung PS, Lee WA
Acta Neurol Taiwan · 2021 Mar · PMID 34549400
PURPOSE: Case presentation of newly diagnosed systemic lupus erythematosus (SLE) presenting initially as Tolosa-Hunt syndrome (THS). STUDY DESIGN: Retrospective clinical case. METHOD: Case report. RESULTS: A healthy youn...
PURPOSE: Case presentation of newly diagnosed systemic lupus erythematosus (SLE) presenting initially as Tolosa-Hunt syndrome (THS). STUDY DESIGN: Retrospective clinical case. METHOD: Case report. RESULTS: A healthy young man developed acute binocular diplopia within 2 days without other neurological deficits. Bilateral 6th cranial nerve palsy was observed with general reduction in the visual field test. Emergent brain magnetic resonance image (MRI) was performed, which revealed severe inflammation in the cavernous sinus, superior orbital fissure, and apex of the orbit. No cavernous thrombosis or intracranial lesion was shown in the MRI. THS was diagnosed and the patient's CN 6 palsy recovered quickly after corticosteroid treatment. However, severe anaemia was discovered during admission (Hb=6.0), so the patient was evaluated by profound laboratory tests, which revealed SLE. CONCLUSION: With painful ophthalmoplegia, cavernous sinus syndrome is highly suspected. THS is one of the differential diagnoses for cavernous sinus syndrome. THS is a rare disease, recognized by the National Organization for Rare Disorders, and characterized by inflammatory changes in the cavernous sinus, superior orbital fissure and/or orbital apex under image study. The inflammatory changes are mostly idiopathic, but secondary causes such as sarcoidosis or other autoimmune diseases need to be ruled out. Physicians should be aware of possible underlying conditions, such as immunosuppressed status as in SLE, as the true cause of THS.
Pain Relief in Short-Lasting Unilateral Neuralgiform Headache with Conjunctival inJection and Tearing Syndrome with Intravenous Ketamine: A Case Report.
Shiiba S, Sago T, Kawabata K
Acta Neurol Taiwan · 2021 Mar · PMID 34549399
PURPOSE: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare form of primary headache, classified as trigeminal autonomic cephalalgia. Since the underlying mechanism o...
PURPOSE: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare form of primary headache, classified as trigeminal autonomic cephalalgia. Since the underlying mechanism of the pathogenesis has not yet been determined, a standardized therapeutic strategy for SUNCT is unavailable. We present a case of SUNCT syndrome with successful pain relief by intravenous administration of ketamine, an N-methyl-D-aspartate receptor (NMDAR) antagonist. CASE REPORT: A 56-year-old male patient reported severe throbbing and shooting pain in forehead, temporal and periorbital region. We confirmed conjunctival injection, lacrimation, blepharoptosis, and miosis as symptoms related to autonomic activity, and made a diagnosis of SUNCT based on ICHD-3 beta. Numerous treatments were attempted, including pregabalin, gabapentine, nonsteroidal antiinflammatory drugs, acetaminophen, steroids, antidepressants, triptans, nerve blocks, and intravenous lidocaine with unsatisfactory results. Intravenous administration of ketamine (0.4 mg/kg) for one hour, was found to relieve the severe pain. CONCLUSION: Intravenous ketamine can effectively treat SUNCT syndrome. This case demonstrated that involvement of NMDAR could be one of the mechanisms of SUNCT syndrome pathogenesis and establish a therapeutic strategy for this pain syndrome.
Association of COVID-19 Infection and Juvenile Stroke: a Case Series.
Mozhdehipanah H, Paybast S, Mavandadi S
Acta Neurol Taiwan · 2021 Mar · PMID 34549398
Ischemic stroke has been increasingly reported as a consequence of COVID-19 infection. However, the underlying etiology is not well determined. The objective of this study is to discuss association of juvenile stroke wit...
Ischemic stroke has been increasingly reported as a consequence of COVID-19 infection. However, the underlying etiology is not well determined. The objective of this study is to discuss association of juvenile stroke with COVID-19 infection. We analyzed 5 COVID-19 positive and stroke patients with a mean age of 41.2 years-old. Three patients developed large vessel occlusion, one small vessel occlusion and one PRES with superimposed lobar ICH, respectively. The mean initial NIHSS of our patients was 11.6. Except the one with massive cerebellar infarct, a desirable outcome occurred with a mean mRS 2.6 at discharge. The mean ESR and CRP level was elevated to 30.4 ml and 32 mg/dl. The severity of COVID-19 infection was considered mainly as mild. COVID-19 infection has the potential to induce hypercoagulability state contributing to stroke development even in the mild form of disease. Keywords: Cerebrovascular Accident, CVA, Stroke, COVID-19, Novel Coronavirus Acta.
Human Herpes Virus 6 Encephalitis Presenting as Fatal Refractory Status Epilepticus: a Case Report and Systematic Review.
Ocampo FF, Tipones RN, Jamora RDG
Acta Neurol Taiwan · 2021 Mar · PMID 34549397
PURPOSE: Encephalitis secondary to human herpesvirus 6 (HHV-6) infection is frequently encountered in immunocompromised patients; in contrast, HHV-6 encephalitis in immunocompetent patients is rare. There are only 3 repo...
PURPOSE: Encephalitis secondary to human herpesvirus 6 (HHV-6) infection is frequently encountered in immunocompromised patients; in contrast, HHV-6 encephalitis in immunocompetent patients is rare. There are only 3 reports of status epilepticus due to HHV-6 encephalitis in immunocompetent adults. In the present study, a case of refractory status epilepticus secondary to HHV-6 encephalitis was reported in an immunocompetent female. CASE REPORT: We report a case of a previously healthy 46-year-old female who presented with a one-week history of back pain, fever and generalized tonic-clonic seizures that progressed to status epilepticus. The video electroencephalography showed epileptiform discharges on both frontotemporal regions. Neuroimaging showed hyperintensities on the bilateral insula and temporal lobes. The cerebrospinal fluid showed elevated pressure and was positive for HHV-6. She was given ganciclovir and a total of eleven antiepileptic drugs. Despite these medications, she developed refractory status epilepticus and eventually succumbed due to multiple medical complications. CONCLUSION: This case highlights HHV-6 encephalitis as an important diagnostic consideration in patients presenting with refractory status epilepticus, regardless of immune status.
The Clinical Features and Therapeutic Outcomes of Young Adults with Cryptococcal Meningitis.
Huang YJ, Tsai WC, Lien CY … +3 more , Lee JJ, Lu CH, Chang WN
Acta Neurol Taiwan · 2021 Mar · PMID 34549396
OBJECTIVE: No previous study has reported on the clinical characteristics of cryptococcal meningitis (CM) focusing solely on young adults. PATIENTS AND METHODS: Ninety-nine adult patients with CM (64 men and 35 women) we...
OBJECTIVE: No previous study has reported on the clinical characteristics of cryptococcal meningitis (CM) focusing solely on young adults. PATIENTS AND METHODS: Ninety-nine adult patients with CM (64 men and 35 women) were enrolled, of whom 26 were classified into the young adult group (≤ 40 years) and 73 into the non-young adult group (> 40 years). The modified Rankin scale (mRS) was used to evaluate the outcomes of the survivors at the time of discharge and at 1 year of follow-up. The clinical characteristics and laboratory data between 1) the young adult CM patients with and without acquired immunecompromised syndrome and 2) the male and female young adult CM patients were compared. The prognostic factors of the young adult CM patients were also analyzed. RESULTS: The young adult group had a higher incidence of headache as the clinical presentation which may have been due to the higher intracranial pressure in this group. The overall mortality rate of the young adults with CM was high (38.5%, 10/26), but no significant prognostic factors were found. In followup studies of the neurologic deficits, the young adult survivors had better outcomes (mRS scores = 0-2) than the non-young adult group at discharge and 1 year after discharge. CONCLUSION: The young adult CM patients had a higher incidence of headache as the clinical presentation. Although the mortality rate in the young adult CM patients was high, the survivors had better neurologic outcomes.
Determinants for Control of Status Epilepticus in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis.
Yen HK, Tang SC, Tsai LK … +3 more , Fan SP, Yeh SJ, Jeng JS
Acta Neurol Taiwan · 2021 Mar · PMID 34549395
BACKGROUND: Treatment guideline for status epilepticus (SE) specifically in patients with anti-N-methyl- D-aspartate receptor (anti-NMDAR) encephalitis is insufficient. This study aimed to clarify the determinants for th...
BACKGROUND: Treatment guideline for status epilepticus (SE) specifically in patients with anti-N-methyl- D-aspartate receptor (anti-NMDAR) encephalitis is insufficient. This study aimed to clarify the determinants for the control of SE in adult patients with anti-NMDAR encephalitis. METHODS: Medical records of all patients with anti-NMDAR encephalitis hospitalized between Jan. 2010 and Sep. 2019 were analyzed for the time sequence of seizures and treatments, and antiepileptic drug (AED) regimens related to SE. The outcomes were control of SE and seizures, and the discharge score of modified Rankin Scale (mRS). RESULTS: All eight patients had seizures and seven (87.5%) suffered from SE which lasted for 3.6 ± 3.9 days. Five patients (71.4%) had SE earlier than using IT, whose SE was controlled by AEDs alone (n = 4) or combined with teratomas resection (n = 1). Another two patients suffered from SE after receiving IT, and one of them had SE only for 1 hour. Moreover, all SE patients received increased types and dosages of AEDs at SE end. A shorter duration of refractory SE was associated with its later occurrence after seizure onset (p = 0.005) and longer duration of AEDs use before SE (p = 0.026). All cases achieved seizure freedom after receiving AEDs and IT. CONCLUSIONS: In these patients with anti-NMDAR encephalitis, all the SE which occurred before initiating IT was successfully controlled by AEDs alone or combined with teratoma resection, and later onset of refractory SE was associated with a shorter SE duration.
Multiple Myeloma: Lytic Bone Lesions of the Skull.
Rossi UG, Ierardi AM, Cariati M
Acta Neurol Taiwan · 2021 Jun · PMID 34549394
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 le...
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.
Sympathetic storm or Cytokine storm: A diagnostic dilemma in patient of traumatic brain injury with COVID 19.
Singh K, Kumar N, Kumar A … +2 more , Kumar A, Shaju AR
Acta Neurol Taiwan · 2021 Jun · PMID 34549393
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 sympathe...
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.
Anti-SOX1 Antibody-Positive Paraneoplastic Syndrome Presenting with Subacute Cerebellar Degeneration and Lambert-Eaton Myasthenic Syndrome: A Case Report.
Cheng YC, Chang A, Hsu WC
Acta Neurol Taiwan · 2021 Jun · PMID 34549392
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...
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.
About
- Frequency
- Sun
- Papers found
- 200
- RSS feed
- Subscribe