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Palatal myoclonus

Naresh Mullaguri, Anusha Battineni, Miguel Chuquilin
BACKGROUND: Wernekink commissure syndrome causes a peculiar combination of internuclear ophthalmoplegia, dysarthria, and delayed-onset palatal myoclonus. Palatal myoclonus is thought to be secondary to delayed hypertrophic degeneration of the bilateral inferior olivary nuclei secondary to involvement of bilateral dentatoolivary tract. We describe a case of a patient with early-onset palatal myoclonus. CASE PRESENTATION: A 53-year-old Caucasian man with several vascular risk factors presented to our emergency room with slurred speech, double vision, difficulty with swallowing and walking, and rhythmic contractions of the soft palate...
May 1, 2018: Journal of Medical Case Reports
Bo-Ram Kim, Yejin Lee, Soo Jin Kim, Hyuntae Kim, Jong Won Lee, Seunghwan Lee, Jongmin Lee
Palatal myoclonus (PM) is a rare disease that may induce dysphagia. Since dysphagia related to PM is unique and is characterized by myoclonic movements of the involved muscles, specific treatments are needed for rehabilitation. However, no study has investigated the treatment effectiveness for this condition. Therefore, the aim of this case report was to describe the benefit of combining behavioral treatment with valproic acid administration in patients with dysphagia triggered by PM. The two cases were treated with combined treatment...
February 2018: Annals of Rehabilitation Medicine
Luísa Panadés-de Oliveira, Antonio Méndez-Guerrero, Roberto López-Blanco, Elena Salvador Álvarez, Juan Ruiz Morales, Jesús González de la Aleja
No abstract text is available yet for this article.
February 6, 2018: Neurology
Mehmet Resid Onen, Kelli Moore, Ulas Cikla, Melih Ucer, Bradley Schmidt, Aaron S Field, Mustafa K Baskaya
BACKGROUND: Hypertrophic olivary degeneration (HOD) occurs because of posterior fossa or brainstem lesions that disrupt the dentato-rubro-olivary tract, well known as the Guillain-Mollaret triangle. Clinical and radiologic hallmarks of this condition are palatal myoclonus and T2 hyperintensity of the inferior olivary complex on magnetic resonance imaging (MRI), respectively. Because symptomatic HOD can complicate the recovery of patients with posterior fossa or brainstem lesions, the purpose of this study is to evaluate clinical and imaging findings of patients with HOD...
April 2018: World Neurosurgery
G Heimer, E Eyal, X Zhu, E K Ruzzo, D Marek-Yagel, Doron Sagiv, Y Anikster, H Reznik-Wolf, E Pras, D Oz Levi, D Lancet, B Ben-Zeev, A Nissenkorn
BACKGROUND: AIFM1 encodes a mitochondrial flavoprotein with a dual role (NADH oxidoreductase and regulator of apoptosis), which uses riboflavin as a cofactor. Mutations in the X-linked AIFM1 were reported in relation to two main phenotypes: a severe infantile mitochondrial encephalomyopathy and an early-onset axonal sensorimotor neuropathy with hearing loss. In this paper we report two unrelated males harboring AIFM1 mutations (one of which is novel) who display distinct phenotypes including progressive ataxia which partially improved with riboflavin treatment...
January 2018: European Journal of Paediatric Neurology: EJPN
N V Boiko
The objective of the present literature review was to systematize the available data on the etiology, pathogenesis, clinical features, diagnostics, and treatment of muscular tympanophonia. The commonest source of muscular tympanophonia is the tremor (myoclonus) of the soft palate or the middle ear; sometimes this condition can be a consequence of myoclonus of the external ear muscles or head muscles. Pharmacotherapy of muscular tympanophonia does not invariably result in the favourable outcome. The author describes a rare observation of objective tympanophonia attributable to myoclonus of the auricular muscle...
2017: Vestnik Otorinolaringologii
Stanislas Ballivet de Régloix, A Marhic, Y Pons
No abstract text is available yet for this article.
June 2017: Journal of the Royal Army Medical Corps
Chandra Mohan Sharma, Rajendra Kumar Pandey, Banshi Lal Kumawat, Dinesh Khandelwal, Pankaj Gandhi
Antibodies to GAD-65 have been implicated in the pathogenesis of type 1 diabetes, limbic encephalitis and Stiff person syndrome, however these diseases rarely occur concurrently. We intend to present a rare case of 35 year old female who was recently diagnosed as having type 1 diabetes presented with 1½ month history of recurrent seizures, subacute onset gait ataxia, dysathria, psychiatric disturbance and cognitive decline. No tumor was found on imaging and the classic paraneoplastic panel was negative. Cerebrospinal fluid and blood was positive for GAD-65 antibodies...
January 2016: Annals of Indian Academy of Neurology
Carlos Henrique Carvalho, Hubert Kimmig, William Omar Contreras Lopez, Manfred Lange, Reinhard Oeckler
Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the olivary body, part of the medulla oblongata, representing a major source of input to the cerebellum. HOD typically results from focal lesions interrupting connections from the inferior olive within the dentato-rubro-olivary pathway, a region also known as the triangle of Guillain-Mollaret (TGM) (red nucleus, inferior olivary nucleus, and contralateral dentate nucleus)...
January 2016: Journal of Neurological Surgery. Part A, Central European Neurosurgery
Heather R Minkel, Tooba Z Anwer, Kara M Arps, Michael Brenner, Michelle L Olsen
Alexander Disease (AxD) is a "gliopathy" caused by toxic, dominant gain-of-function mutations in the glial fibrillary acidic protein (GFAP) gene. Two distinct types of AxD exist. Type I AxD affected individuals develop cerebral symptoms by 4 years of age and suffer from macrocephaly, seizures, and physical and mental delays. As detection and diagnosis have improved, approximately half of all AxD patients diagnosed have onset >4 years and brainstem/spinal cord involvement. Type II AxD patients experience ataxia, palatal myoclonus, dysphagia, and dysphonia...
December 2015: Glia
Sanjeev Sivakumar, Kumar Rajamani
A 59-year-old woman with a history of diabetes, hypertension, and multiple previous strokes presented for evaluation. Two years earlier, she had an acute onset of imbalance, along with difficulty swallowing and speaking. The neurologic examination revealed dysarthric speech and pseudobulbar affect,..
July 16, 2015: New England Journal of Medicine
Raheel Ahmed, Arnold H Menezes
Clinical presentation of craniovertebral junction disorders may range from acute catastrophic neurological deficits to insidious signs and symptoms that may mask the underlying etiology. Prompt recognition and treatment is essential to avert long-term neurological morbidity. Proatlas segmentation disorders are a rare group of developmental disorders involving the craniocervical junction. Abnormal bony segmentation leads to malformed bony structures that can in turn lead to neurological deficits through bony compression of the cervicomedullary junction...
September 2015: Journal of Neurosurgery. Pediatrics
Sara Wein, Bernard Yan, Frank Gaillard
We report a 58-year-old man who developed hyptertrophic olivary degeneration (HOD) after haemorrhage of a cavernous malformation in the pons. Lesions of the triangle of Guillain and Mollaret (the dentatorubro-olivary pathway) may lead to HOD, a secondary transsynaptic degeneration of the inferior olivary nucleus. HOD is considered unique because the degenerating olive initially becomes hypertrophic rather than atrophic. The primary lesion causing pathway interruption is often haemorrhage, either due to hypertension, trauma, surgery or, as in our patient, a vascular malformation such as a cavernoma...
July 2015: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Paolo Immovilli, Eugenia Rota, Nicola Morelli, Ilaria Iafelice, Andrea Magnacavallo, Donata Guidetti
No abstract text is available yet for this article.
March 24, 2015: Neurology
José Fidel Baizabal-Carvallo, Robert Fekete
BACKGROUND: Psychogenic or functional movement disorders (PMDs) pose a challenge in clinical diagnosis. There are several clues, including sudden onset, incongruous symptoms, distractibility, suggestibility, entrainment of symptoms, and lack of response to otherwise effective pharmacological therapies, that help identify the most common psychogenic movements such as tremor, dystonia, and myoclonus. METHODS: In this manuscript, we review the frequency, distinct clinical features, functional imaging, and neurophysiological tests that can help in the diagnosis of uncommon presentations of PMDs, such as psychogenic parkinsonism, tics, and chorea; facial, palatal, and ocular movements are also reviewed...
2015: Tremor and Other Hyperkinetic Movements
Djalma F S Menéndez, Rubens G Cury, Egberto R Barbosa, Manoel J Teixeira, Erich T Fonoff
BACKGROUND: Hypertrophic olivary degeneration (HOD) is a rare phenomenon, probably related to transsynaptic degeneration of the inferior olivary nucleus. It usually occurs as a response to primary injury of dento-rubro-olivary pathways. CASE REPORT: A young man developed Holmes' tremor 7 months after a cavernous malformation bleed in the midbrain. Typical findings of HOD were observed in the magnetic resonance images: bilateral and asymmetric hypertrophy of the olivary nucleus with slight hypersignal in T2-weighted images...
2014: Tremor and Other Hyperkinetic Movements
Andrea Nath Shogan, Derek J Rogers, Christopher J Hartnick, Joseph E Kerschner
The use of botulinum toxin in adult otolaryngology has been commonly used in conditions such as spasmodic dysphonia, cricopharyngeal spasm, palatal myoclonus, sialorrhea, and for cosmetic reasons. The current use of botulinum toxin in pediatric otolaryngology and laryngology has primarily been off label and in children older than 2 years of age. This review discusses the different applications of botulinum toxin in pediatric patients and its effectiveness in treating different pediatric conditions.
September 2014: International Journal of Pediatric Otorhinolaryngology
Bilge Tuna, Mehmet Hamdi Şahan
Palatal myoclonus is an extremely rare disorder. Tinnitus is secondary to rhythmic involuntary movements of the soft palate. Clinical diagnosis is based on the confirmation of the soft palate movements synchronous with an audible clicking noise outside. In this article, we report a 38-year-old female case with a 10 year-history of continuing ear click, who was diagnosed with essential palatal myoclonus. The disease and its differential diagnosis from symptomatic palatal myoclonus were discussed in the light of literature data...
January 2014: Kulak Burun Boğaz Ihtisas Dergisi: KBB, Journal of Ear, Nose, and Throat
Catherine F Sinclair, Lowell E Gurey, Andrew Blitzer
OBJECTIVES/HYPOTHESIS: To review the clinical characteristics and management of patients with palatal myoclonus and devise an algorithm for treatment with botulinum toxin based on presenting symptoms, clinical examination findings, and involved muscle groups. STUDY DESIGN: Retrospective chart review at two clinical research centers. METHODS: Between 1985 and 2011, 15 patients with a diagnosis of essential palatal myoclonus were assessed. Data were collected on patient demographics, disease characteristics, and treatment outcomes...
May 2014: Laryngoscope
Esmaeel Ghoreishi, Gholam Ali Shahidi, Mohammad Rohani, Mohammad Nabavi, Mahbubeh Aghaei, Fahimeh Haji Akhoundi
OBJECTIVE: Hashimoto encephalopathy (HE) is known as a steroid- responsive encephalopathy associated with autoimmune thyroiditis or nonvascular inflammation-related autoimmune meningoencephalitis. The average age of onset of HE is approximately 50 years; and it is more common in women. The onset of HE may be acute or subacute. The course of most HE cases is relapsing and remitting, which is similar to that of vasculitis and stroke. METHODS: In this article, we present a previously healthy 32 years old;veterinarian male with palatal myoclonus, as a rare presentation of this disorder, and review the neurologic aspects of hashimoto encephalitis...
August 2013: Iranian Journal of Psychiatry
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