Palatal tremor as a presenting symptom of amyotrophic lateral sclerosis.

Published on Apr 24, 2018in Neurology9.91
路 DOI :10.1212/WNL.0000000000005363
Amir-Hadi Maghzi20
Estimated H-index: 20
(Cedars-Sinai Medical Center),
Supreet K. Sahai3
Estimated H-index: 3
(Cedars-Sinai Medical Center)
+ 1 AuthorsRobert H. Baloh55
Estimated H-index: 55
(Cedars-Sinai Medical Center)
Sources
Abstract
Palatal tremor, also known as palatal myoclonus, is a sign caused by a lesion to the Guillain-Mollaret triangle. In some cases, imaging may also reveal hypertrophic olivary degeneration, which is due to trans-synaptic degeneration and gliosis of the inferior olivary nucleus. The underlying etiology is commonly due to unilateral involvement of the central tegmental tract due to ischemic, neoplastic, demyelinating, traumatic, inflammatory, and rarely neurodegenerative processes such as progressive supranuclear palsy, multiple system atrophy, and Alexander disease.1 We describe a patient who presented with palatal tremor as the presenting symptom of amyotrophic lateral sclerosis (ALS).
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#1B. Echeveste (University of Navarra)H-Index: 3
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Last. Maria-Rosario LuquinH-Index: 25
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#1Sarah Kang (Case Western Reserve University)H-Index: 1
#2Aasef G. Shaikh (Case Western Reserve University)H-Index: 23
Acquired pendular nystagmus is comprised of quasi-sinusoidal oscillations of the eyes significantly affecting gaze holding and clarity of vision. The most common causes of acquired pendular nystagmus include demyelinating disorders such as multiple sclerosis and the syndrome of ocular palatal tremor. However, several other deficits, such as pharmacological intoxication, metabolic and genetic disorders, and granulomatous disorders can lead to syndromes mimicking acquired pendular nystagmus. Study...
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#1Leo McCluskey (UPenn: University of Pennsylvania)H-Index: 61
#2Shannon Vandriel (UPenn: University of Pennsylvania)H-Index: 1
Last. Murray Grossman (UPenn: University of Pennsylvania)H-Index: 116
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Abstract Objective Autopsy studies show widespread pathology in amyotrophic lateral sclerosis (ALS), but clinical surveys of multisystem disease in ALS are rare. We investigated ALS-Plus syndrome, an understudied group of patients with clinical features extending beyond pyramidal and neuromuscular systems with or without cognitive/behavioral deficits. Methods In a large, consecutively-ascertained cohort of 550 patients with ALS, we documented atypical clinical manifestations. Genetic screening f...
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#1Carlo CivardiH-Index: 18
#2Alessandra ColliniH-Index: 5
Last. Francesco MonacoH-Index: 43
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#1Elsdon Storey (Monash University)H-Index: 50
#2Melanie A. Knight (University of Melbourne)H-Index: 20
Last. R. J. McKinlay GardnerH-Index: 23
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Spinocerebellar ataxia type 20 (SCA20) was reported in 2004 in a single Australian Anglo-Celtic pedigree. The phenotype is distinctive, with palatal tremor, and hypermetric saccades, and early dentate (but not pallidal) calcification in the absence of abnormalities of calcium metabolism. Dysarthria, rather than gait ataxia, was the initial symptom in most, and was typically conjoined with dysphonia, clinically resembling adductor spasmodic dysphonia. The onset of these speech abnormalities was a...
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#1Michael Samuel (University of Cambridge)H-Index: 24
#2Nurhan Torun (U of T: University of Toronto)H-Index: 11
Last. Anthony E. Lang (U of T: University of Toronto)H-Index: 168
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Palatal tremor has been subdivided into essential (EPT) and symptomatic palatal tremor (SPT). A subgroup of the SPT form has a syndrome of progressive ataxia and palatal tremor (PAPT). Published details of cases of PAPT are sparse and the disorder appears heterogeneous. We present clinical and MRI features of six patients with sporadic PAPT who attended The University Health Network between 1991 and 2002. Eye movements were recorded using a magnetic search coil technique. We review previously re...
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Neuroferritinopathy is a recently described autosomal dominant, adult onset, neurodegenerative disorder associated with iron accumulation, particularly in the basal ganglia.1 All patients found to date have a single adenine insertion between nucleotides 460 and 461 in exon 4 of the ferritin light chain gene. This results in a frame shift and is predicted to cause structural alteration of the polypeptide carboxy terminus. Magnetic resonance imaging of the brain shows iron accumulation, and this h...
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Background: Palatal tremor is involuntary, rhythmic and oscillatory movement of the soft palate. Palatal tremor can be classified into three subtypes; essential, symptomatic and palatal tremor associated with progressive ataxia. Methods: A thorough Pubmed search was conducted to look for the original articles, reviews, letters to editor, case reports, and teaching neuroimages, with the keywords 鈥渆ssential鈥, 鈥渟ymptomatic palatal tremor鈥, 鈥渕yoclonus鈥, 鈥渁taxia鈥, 鈥渉ypertrophic鈥, 鈥渙livary鈥 and 鈥渄egen...
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