- Ipsilateral hypoglossal nerve palsy following left hemithyroidectomy: Case report and review of literature. [Journal Article]
- IJInt J Surg Case Rep 2018 Aug 11; 51:5-7
- CONCLUSIONS: Few cases of HNP are reported in the literature following oropharyngeal manipulation. Factors such as the type of surgery, position changes, and intubation characteristics have been linked to the incidence of HNP. Most of the cases recovered spontaneously, indicating a neuro-paxic type of injury. One case of HNP was reported following robotic total thyroidectomy, which was referred to as iatrogenic complication, and resulted in permanent paralysis. We believe that our case is the only reported case of transient ipsilateral HNP following a conventional left hemithyroidectomy.Strap muscles retraction and neck extension during thyroidectomy could predispose to HNP.
- Purkinje cell COX deficiency and mtDNA depletion in an animal model of spinocerebellar ataxia type 1. [Journal Article]
- JNJ Neurosci Res 2018; 96(9):1576-1585
- Spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of cerebellar degenerative disorders, characterized by progressive gait unsteadiness, hand incoordination, and dysarthria. Ataxia ...
Spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of cerebellar degenerative disorders, characterized by progressive gait unsteadiness, hand incoordination, and dysarthria. Ataxia type 1 (SCA1) is caused by the expansion of a CAG trinucleotide repeat in the SCA1 gene resulting in the atypical extension of a polyglutamine (polyQ) tract within the ataxin-1 protein. Our main objective was to investigate the mitochondrial oxidative metabolism in the cerebellum of transgenic SCA1 mice. SCA1 transgenic mice develop clinical features in the early life stages (around 5 weeks of age) presenting pathological cerebellar signs with concomitant progressive Purkinje neuron atrophy and relatively little cell loss; this evidence suggests that the SCA1 phenotype is not the result of cell death per se, but a possible effect of cellular dysfunction that occurs before neuronal demise. We studied the mitochondrial oxidative metabolism in cerebellar cells from both homozygous and heterozygous transgenic SCA1 mice, aged 2 and 6 months. Histochemical examination showed a cytochrome-c-oxidase (COX) deficiency in the Purkinje cells (PCs) of both heterozygous and homozygous mice, the oxidative defect being more prominent in older mice, in which the percentage of COX-deficient PC was up to 30%. Using a laser-microdissector, we evaluated the mitochondrial DNA (mtDNA) content on selectively isolated COX-competent and COX-deficient PC by quantitative Polymerase Chain Reaction and we found mtDNA depletion in those with oxidative dysfunction. In conclusion, the selective oxidative metabolism defect observed in neuronal PC expressing mutant ataxin occurs as early as 8 weeks of age thus representing an early step in the PC degeneration process in SCA1 disease.
- CIDP, myasthenia gravis, and membranous glomerulonephritis - three autoimmune disorders in one patient: a case report. [Journal Article]
- BNBMC Neurol 2018 Aug 14; 18(1):113
- CONCLUSIONS: All three diseases are of autoimmune origin with distinctive immunopathogenetic mechanisms. The present case of CIDP, MG, and MGN occurring in one patient indicates a common underlying immune mechanism in these distinct conditions, including the involvement of autoantibodies and T cells.
- Communication Matters-Pitfalls and Promise of Hightech Communication Devices in Palliative Care of Severely Physically Disabled Patients With Amyotrophic Lateral Sclerosis. [Review]
- FNFront Neurol 2018; 9:603
- Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease, leading to progressive paralysis, dysarthria, dysphagia, and respiratory disabilities. Therapy is mostly focused on pallia...
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease, leading to progressive paralysis, dysarthria, dysphagia, and respiratory disabilities. Therapy is mostly focused on palliative interventions. During the course of the disease, verbal as well as nonverbal communicative abilities become more and more impaired. In this light, communication has been argued to be "the essence of human life" and crucial for patients' quality of life. High-tech augmentative and alternative communication (HT-AAC) technologies such as eyetracking based computer devices and brain-computer-interfaces provide the possibility to maintain caregiver-independent communication and environmental control even in the advanced disease state of ALS. Thus, they enable patients to preserve social participation and to independently communicate end-of-life-decisions. In accordance with these functions of HT-AAC, their use is reported to strengthen self-determination, increase patients' quality of life and reduce caregiver burden. Therefore, HT-AAC should be considered as standard of (palliative) care for people with ALS. On the other hand, the supply with individually tailored HT-AAC technologies is limited by external and patient-inherent variables. This review aims to provide an overview of the possibilities and limitations of HT-AAC technologies and discuss their role in the palliative care for patients with ALS.
- Integration of a neurodynamic approach into the treatment of dysarthria for patients with idiopathic Parkinson's disease: A pilot study. [Journal Article]
- JBJ Bodyw Mov Ther 2018; 22(3):648-656
- CONCLUSIONS: The significant differences in the pre-post comparison within the groups may indicate a high probability of a positive effect of standard dysarthria treatment on the severity of dysarthria. In between-group comparisons, the study results indicated no evidence of a significant difference between standard dysarthria treatment with or without neurodynamics. Due to the small sample size, the effectiveness of the integration of neurodynamics into speech therapy cannot be definitively concluded for now. In order to be able to have generalized applicability, future studies with larger numbers of participants are required.
- Prevalence and phenotype of the c.1529C>T SPG7 variant in adult-onset cerebellar ataxia in Italy. [Journal Article]
- EJEur J Neurol 2018 Aug 11
- CONCLUSIONS: SPG7 c.1529C>T (p.Ala510Val) mutants account for 2.3% of cerebellar ataxia cases in Italy, suggesting that this variant should be considered as priority test in the presence of late onset pure ataxia. Moreover, the heterozygous/homozygous genotype appears to predict the onset of clinical manifestation and disease progression. This article is protected by copyright. All rights reserved.
- An unusual case of inflammatory meningitis in a young man with systemic lupus erythematosus. [Journal Article]
- LLupus 2018 Aug 09; :961203318791760
- We describe a man presenting with unusual neurological manifestations of systemic lupus erythematosus (SLE) including pachymeningitis, aseptic meningitis and encephalitis with grossly elevated cerebr...
We describe a man presenting with unusual neurological manifestations of systemic lupus erythematosus (SLE) including pachymeningitis, aseptic meningitis and encephalitis with grossly elevated cerebrospinal fluid protein, responding to immunosuppression. Initially he had intermittent dysarthria, dysphasia and unilateral upper limb weakness. One month later he experienced dysphasia, right-sided hemiparesis and confusion. Cerebrospinal fluid (CSF) analysis showed a white cell count of 70 x 106/litre and an unusually elevated protein level of 5.39 g/litre. An MRI brain showed dural and leptomeningeal enhancement compatible with a meningitic process. He improved with cefotaxime and aciclovir. On day seven of antimicrobials he developed left-sided weakness, sensory inattention and a left homonymous hemianopia. He responded well to intravenous methylprednisolone. On switching to oral prednisolone he developed expressive dysphasia, a right inferior quadrantanopia and seizures. His bloods were suggestive of macrophage activation syndrome. The patient improved with methylprednisolone and intravenous immunoglobulins, and the improvement was sustained on switching back to oral prednisolone. The prevalence of neuropsychiatric manifestations of SLE varies between 14 and 80% and according to the American College of Rheumatology includes 19 conditions. This case is unique because although some features were in keeping with aseptic meningitis the MRI appearances were also suggestive of pachymeningitis.
- Successful endovascular recanalization of a partially occluded basilar artery fenestration. [Journal Article]
- INInterv Neuroradiol 2018 Aug 09; :1591019918793340
- A 76-year-old man with a history of arterial hypertension, obstructive sleep apnea, dyslipidemia, family history of cardiovascular events, prestroke and overweight presented 90 minutes after acute on...
A 76-year-old man with a history of arterial hypertension, obstructive sleep apnea, dyslipidemia, family history of cardiovascular events, prestroke and overweight presented 90 minutes after acute onset of right-sided sensorimotor hemiparesis, hemiataxia and dysarthria (National Institutes of Health Stroke Scale (NIHSS) 9/42). Magnetic resonance imaging (MRI) revealed a pontine ischemia and MR angiography showed a thrombus in the middle to distal portion of the basilar artery. Owing to the location, an occlusion of one lumen of a fenestrated basilar artery was suspected. Fearing the risk of peripheral dislocation, intravenous thrombolysis was withheld after an interdisciplinary discussion and direct endovascular thrombectomy (Solitaire stent retriever) was successfully performed by passing the stent retriever specifically through the affected lumen and between the thrombus and the vascular wall, which would normally be avoided. Angiography after complete reperfusion (Thrombolysis in Cerebral Infarction grade 3) confirmed a fenestration in the middle to distal portion of the basilar artery where the thrombus was initially located (blue and green arrow). Follow-up MRI after 24 hours showed only minimal ischemic damage in the left pontine area, and the patient was discharged home with ambulatory physiotherapy for residual minimal gait disturbance (NIHSS 0).
- Factors Associated with Stroke Misdiagnosis in the Emergency Department: A Retrospective Case-Control Study. [Journal Article]
- NNeuroepidemiology 2018 Aug 09; 51(3-4):123-127
- CONCLUSIONS: Patients with stroke misdiagnosis were commonly FAST-negative with nonspecific symptoms including altered mental status, dizziness and nausea/vomiting often associated with PCS. Improved diagnostic accuracy may increase access to acute therapies.
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- Mitochondrial membrane protein-associated neurodegeneration: a case report and literature review. [Journal Article]
- NNeurocase 2018 Aug 08; :1-5
- Mitochondrial membrane protein-associated neurodegeneration (MPAN) is an autosomal recessive disorder caused by mutation in the C19orf12 gene. We report a compound heterozygous c.[32C>T];[205G>A;424A...
Mitochondrial membrane protein-associated neurodegeneration (MPAN) is an autosomal recessive disorder caused by mutation in the C19orf12 gene. We report a compound heterozygous c.[32C>T];[205G>A;424A>G] (p.[Thr11Met];[Gly69Arg;Lys142Glu]) Czech patient who manifested with right foot dystonia, impaired handwriting, attention deficit, and signs of iron accumulation on brain MRI. Gradually, he developed dysarthria, spastic-dystonic gait, pedes cavi, and atrophy of leg muscles. Additionally, we report demographic parameters, clinical signs, and allelic frequencies of C19orf12 mutations of all published MPAN cases. We compared the most frequent mutations, p.Thr11Met and p.Gly69ArgfsX10; the latter was associated with younger age at onset and more frequent optic atrophy in homozygotes.