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Masafumi Hirato, Takaaki Miyagishima, Akio Takahashi, Yuhei Yoshimoto
OBJECTIVE: Long-term effectiveness of selective ventralis intermedius (VIM)-ventralis oralis (VO) thalamotomy with depth microrecording for the treatment of focal dystonia was evaluated. The optimal thalamic areas for controlling focal dystonia were studied based on the electrophysiological and anatomical data. METHODS: Stereotactic selective VIM-VO thalamotomy with depth microrecording was carried out in 8 patients with focal arm and hand dystonia and in one patient with cervical dystonia...
June 14, 2018: World Neurosurgery
Naoko Kurahashi, Yukiko Futamura, Norie Nonobe, Shunsuke Ogaya, Yuki Maki, Ikuko Yoshimura, Takeshi Suzuki, Yosuke Hosokawa, Keitaro Yamada, Kosaburo Aso, Koichi Maruyama, Miho Nakamura
BACKGROUND: There are few studies on hiragana reading skill and phonological awareness in Japanese schoolchildren with periventricular leukomalacia (PVL). METHODS: Three seven-year-old children with PVL who had no intellectual disabilities or dysarthria were recruited. Their perinatal information, brain magnetic resonance image (MRI) at term equivalent age, accompanying neurodevelopmental disorders, ophthalmologic features, Kaufman Assessment Battery for Children (K-ABC), a hiragana reading test (four tasks), and a phonological awareness task (mora reversal tasks) were analyzed...
June 13, 2018: Brain & Development
Amaia Muñoz-Lopetegi, Gorka Fernández García de Eulate, Jon Rodríguez-Antigüedad Muñoz, Alberto Bergareche, Juan José Poza
Opercular myoclonic-anarthric status epilepticus (OMASE) is a rare form of epilepsia partialis continua presenting as fluctuating dysarthria, or even anarthria. The condition is caused by an epileptogenic lesion involving the opercular cortex of either hemisphere. Speech impairment is secondary to bilateral epileptic activity affecting the glossopharyngeal muscles. This bilateral nature of the condition is due to the fact that innervation of cranial nerves V, VII, IX, X and XII from the opercular area of the primary motor cortex is bilateral...
June 15, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
Mario Manto
More than a century after the description of its cardinal components, the cerebellar motor syndrome (CMS) remains a cornerstone of daily clinical ataxiology, in both children and adults. Anatomically, motor cerebellum involves lobules I-V, VI, and VIII. CMS is typically associated with errors in the metrics of voluntary movements and a lack of coordination. Symptoms and motor signs consist of speech deficits, impairments of limb movements, and abnormalities of posture/gait. Ataxic dysarthria has a typical scanning (explosive with staccato) feature, voice has a nasal character, and speech is slurred...
2018: Handbook of Clinical Neurology
Kiera N Berggren, Man Hung, Melissa M Dixon, Jerry Bounsanga, Becky Crockett, Mary D Foye, Yushan Gu, Craig Campbell, Russell J Butterfield, Nicholas E Johnson
INTRODUCTION: We describe an exploratory study of orofacial function in children with congenital myotonic dystrophy (CDM) versus healthy controls. METHODS: We evaluated 41 children with CDM and 29 healthy controls for speech and swallow function and for lingual and labial strength. RESULTS: The Iowa Oral Performance Instrument (IOPI), measuring tongue strength, and a lip force meter (LFM), measuring lip strength, had excellent inter-rater reliability with ICCs of 0...
June 14, 2018: Muscle & Nerve
Hyun-Jung Lee, Sung Hae Chang, Eun Ha Kang, Yun Jong Lee, Yeong Wook Song, You-Jung Ha
Neuromyelitis optica is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) that predominantly affects the optic nerves and spinal cord. With the discovery of the pathogenic anti-aquaporin-4 (AQP4) antibody, the disease was recognized as part of a spectrum of autoimmune diseases that target AQP4, collectively referred to as neuromyelitis optica spectrum disorder (NMOSD). NMOSD consists of conditions that affect various parts of the CNS with the AQP4 antibody. In this article, we report a 43-year-old female patient who was initially diagnosed with primary Sjögren's syndrome (pSS) with CNS involvement, but was later diagnosed with overlapping pSS and NMOSD, which required more intensive treatment...
December 2017: Archives of Rheumatology
Camila Nascimento Alves, Tiago Kiyoshi Kitabayashi Braga, Danusa Neves Somensi, Bruno Sérgio Vilhena do Nascimento, José Antônio Santos de Lima, Satomi Fujihara
The X-linked spinal and bulbar muscular atrophy (Kennedy's disease) is a rare X-linked, recessive, lower motor neuron disease, characterized by weakness, atrophy, and fasciculations of the appendicular and bulbar muscle. The disease is caused by an expansion of the CAG repetition in the androgen receptor gene. Patients with Kennedy's disease have more than 39 CAG repetitions. We report a case of 57-year-old man, resident of Monte Dourado (PA, Brazil) who complained of brachiocrural paresis evolving for 3 years along with fasciculations and tremors of extremities...
June 7, 2018: Einstein
Brian W Wakefield, Crystal M C Masterson, Manuel T Borges, K Joseph Hurt
Stroke and hepatic vein thrombosis are highly associated with neoplasia but are extremely rare events in young, pregnant women. Rare and recurrent thrombotic events in pregnancy increase the suspicion for occult malignancy. We describe the case of a healthy 31-year-old G2P1 who presented with visual changes and dysarthria during pregnancy. Imaging showed cerebral infarcts. Her thrombophilia evaluation was negative. During delivery, she was diagnosed with fulminant Budd-Chiari Syndrome. Hepatic ultrasound suggested malignancy or metastasis, and postpartum CT scan and biopsy confirmed the diagnosis of Stage IV pancreatic cancer...
June 8, 2018: Gynecologic and Obstetric Investigation
Josef Finsterer, Claudia Stöllberger, Chen-Yu Ho
BACKGROUND: Takotsubo syndrome (TTS) is a non-ischemic cardiomyopathy with sudden but transient systolic dysfunction. TTS mimics myocardial infarction clinically, chemically, and electrocardiographically but echocardiography typically shows apical ballooning and coronary angiography is normal. TTS has not been reported in a patient with myasthenia gravis (MG) and polymyositis due to a malignant thymoma. CASE REPORT: Two weeks prior to admission, a 76yo female developed dysarthria, chronic coughing, and disabling myalgias of the entire musculature...
June 8, 2018: International Journal of Neuroscience
Thomas Mathew, Amrutha Avati, Delon D'Souza, Manjusha Therambil
Pontocerebellar hypoplasia type 6 (PCH6) is an autosomal recessive mitochondrial disease, typically characterized by pontine atrophy, vermian hypoplasia, infantile encephalopathy, generalized hypotonia, and intractable seizures. The purpose of this study is to describe the seizures and other neurological manifestations of RARS 2 gene mutations and to compare the clinical features with other causes of progressive myoclonic epilepsy. Detailed history, physical examination, and clinical and genetic work-up were performed in 2 siblings who presented with progressive myoclonic epilepsy...
June 2018: Epilepsia Open
Rachel Quinn, Stacie Park, Deborah Theodoros, Anne J Hill
PURPOSE: This study aimed to determine the feasibility of delivering a group speech maintenance programme (eLoud and Proud) to people with Parkinson's disease via telerehabilitation. METHOD: Treatment was delivered to eight participants who had previously received LSVT LOUD®. The programme focussed on using a "loud" voice within conversational and cognitively loaded tasks, and was delivered in two 90-minute sessions per week for four weeks. Data pertaining to sound pressure level (SPL) (for sustained phonation, reading and monologue tasks), maximum frequency range, maximum phonation duration and impact of dysarthria on quality of life were collected at three time points: (1) pre-treatment (PRE); (2) immediately post-treatment (POST); and (3) three months post-treatment (FU)...
June 7, 2018: International Journal of Speech-language Pathology
Stephanie A Borrie, Kaitlin L Lansford, Tyson S Barrett
A positive relationship between rhythm perception and improved understanding of a naturally dysrhythmic speech signal, ataxic dysarthria, has been previously reported [Borrie, Lansford, and Barrett. (2017). J. Speech Lang. Hear. Res. 60, 3110-3117]. The current follow-on investigation suggests that this relationship depends on the nature of the dysrhythmia. When the corrupted rhythm cues are relatively predictable, affording some learnable acoustic regularity, the relationship is replicated. However, this relationship is nonexistent, along with any intelligibility improvements, when the corrupted rhythm cues are unpredictable...
May 2018: Journal of the Acoustical Society of America
Maija Koivu, Antti Huotarinen, Filip Scheperjans, Aki Laakso, Riku Kivisaari, Eero Pekkonen
OBJECTIVES: To evaluate the efficacy and adverse effects of subthalamic deep brain stimulation (STN-DBS) in patients with advanced Parkinson's disease (PD) and the possible correlation between electrode location and clinical outcome. METHODS: We retrospectively reviewed 87 PD-related STN-DBS operations at Helsinki University Hospital (HUH) from 2007 to 2014. The changes of Unified Parkinson's Disease Rating Scale (UPDRS) part III score, Hoehn & Yahr stage, antiparkinson medication, and adverse effects were studied...
May 30, 2018: Brain and Behavior
Daniel Z Adams, Andrew King, Colin Kaide
We describe a case of wound botulism initially thought to represent Miller-Fisher variant Guillain-Barré syndrome (MFS). Botulism classically presents with the so-called "four D's" (diplopia, dysarthria, dysphagia, dry mouth) with symmetric, descending weakness. MFS presents with a triad of limb-ataxia, areflexia, and ophthalmoplegia, with variable cranial nerve and extremity involvement. The distinction can be difficult but is important as early initiation of botulinum antitoxin is associated with improved patient outcomes in cases of botulism...
August 2017: Clinical practice and cases in emergency medicine
Angela T Morgan, Elizabeth Murray, Frederique J Liégeois
BACKGROUND: Childhood apraxia of speech (CAS) affects a child's ability to produce sounds and syllables precisely and consistently, and to produce words and sentences with accuracy and correct speech rhythm. It is a rare condition, affecting only 0.1% of the general population. Consensus has been reached that three core features have diagnostic validity: (1) inconsistent error production on both consonants and vowels across repeated productions of syllables or words; (2) lengthened and impaired coarticulatory transitions between sounds and syllables; and (3) inappropriate prosody (ASHA 2007)...
May 30, 2018: Cochrane Database of Systematic Reviews
Bianca Simone Zeigelboim, Hélio A G Teive, Geslaine Janaína Barbosa Santos, Maria Izabel Rodrigues Severiano, Vinicius Ribas Fonseca, João Henrique Faryniuk, Jair Mendes Marques
Objective To describe and compare the vestibular findings most evident among the hereditary ataxias, as well as correlate their clinical features with the nervous structures affected in this disease. Methods Seventy-five patients were evaluated and underwent a case history, otorhinolaryngological and vestibular assessments. Results Clinically, the patients commonly had symptoms of gait disturbances (67.1%), dizziness (47.3%), dysarthria (46%) and dysphagia (36.8%). In vestibular testing, alterations were predominantly evident in caloric testing (79%), testing for saccadic dysmetria (51%) and rotational chair testing (47%)...
March 2018: Arquivos de Neuro-psiquiatria
Chikezie Alvarez, Hafiz Muhammad Aslam, Sara Wallach, Muhammad U Mustafa
Aortic atheromas (aortic atheromatous plaques) are defined by an irregular thickening of the intima ≥2 mm, and a complex plaque is defined as a protruding atheroma ≥4 mm with or without an attached mobile component. Stroke incidence is approximately 25% in patients with mobile plaques of the aortic arch and 2% in patients with quiescent nonmobile plaques. Antiplatelet agents, oral anticoagulants, and statins have been suggested in the management of atheromas. We present an 80-year-old male, with non-ST-segment elevation myocardial infarction (NSTEMI) and chronic dysarthria, found to have an acute cerebrovascular accident (CVA) secondary to embolism from a large 12 mm aortic arch plaque, treated medically with oral antiplatelet therapy, anticoagulation, and statin therapy...
2018: Case Reports in Medicine
Yoshiyasu Matsumoto, Hiroshi Kashimura, Kenta Aso, Hiroaki Saura, Mitsumasa Osakabe, Akira Kurose
BACKGROUND: Hemorrhage at presentation in primary central nervous system (CNS) lymphoma is rare. We encountered a case of primary CNS lymphoma presenting as a growing intracerebral hemorrhage. CASE DESCRIPTION: An 80-year-old man presented with mild dysarthria. Computed tomography (CT) demonstrated a round, high-density mass with surrounding vasogenic edema in the left frontal lobe. Although the patient was placed on antihypertensive therapy for suspected subacute subcortical hemorrhage, neurological symptoms gradually worsened...
May 23, 2018: World Neurosurgery
Jimin Lee, Michael Bell
Purpose: The current study examined overall articulatory range of movement (ROM) in individuals with amyotrophic lateral sclerosis (ALS). Differential involvement of articulators was also tested using articulatory working space in individuals with varying degrees of dysarthria severity and in typically aging individuals. A strong association between overall articulatory ROM and severity measures among individuals with ALS was hypothesized. In addition, it was hypothesized that differential involvement of articulators would be detected using overall articulatory ROM measures...
May 24, 2018: American Journal of Speech-language Pathology
Katerina A Tetzloff, Joseph R Duffy, Edythe A Strand, Mary M Machulda, Sarah M Boland, Rene L Utianski, Hugo Botha, Matthew L Senjem, Christopher G Schwarz, Keith A Josephs, Jennifer L Whitwell
Primary progressive apraxia of speech (PPAOS) is a neurodegenerative disorder in which AOS is the sole presenting complaint. We report clinical and neuroimaging data spanning 10 years from disease onset-to-death in a 49 year-old male PPAOS patient, DY, who died with corticobasal degeneration. He presented with AOS with normal neuroimaging. Abnormalities in the caudate nucleus, supplementary motor area, cingulate, insula, and Broca's area were observed after five years, with involvement of motor cortex and development of agrammatism, Parkinsonism, and dysarthria three years later...
May 25, 2018: Neurocase
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