A previously unreported association of amyotrophic lateral sclerosis and ocular flutter is presented. It is hypothesized that initial loss of brainstem inhibitory interneurons resulted in disinhibition of burst interneurons and that the ocular flutter subsequently disappeared as burst interneurons also became affected by the disease process. The association adds clinical evidence of involvement of brainstem interneurons to other evidence of involvement of neurons other than motor neurons in the disease process.
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http://dx.doi.org/10.3109/17482960902875133 | DOI Listing |
Acta Neurol Belg
December 2024
Department of Pediatrics, University of Health Sciences, Kartal Dr. Lutfi Kirdar City Hospital, Istanbul, Turkey.
Intern Med J
September 2024
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
J Pediatr
December 2024
Division of Child Neurology, Department of Neurology and Neurological Sciences, Stanford University, Palo Alto, CA; Division of Neuro-Ophthalmology, Department of Neurology and Neurological Sciences, Stanford University, Palo Alto, CA.
Tremor Other Hyperkinet Mov (N Y)
July 2024
Neurology Service, University Hospital Marqués de Valdecilla-IDIVAL, Santander, Spain.
Background: Severe hypomagnesemia is an increasingly recognized cause of acute and reversible cerebellar ataxia, often accompanied by cerebellar oculomotor signs such as jerky horizontal or downbeat nystagmus and very rarely ocular flutter.
Phenomenology Shown: This video illustrates horizontal pendular nystagmus in a patient with acute onset cerebellar ataxia associated with severe hypomagnesemia.
Educational Value: Acquired pendular nystagmus can be distinguished from macrosaccadic oscillations and ocular flutter in that the former is composed of two slow phases of equal velocity and the latter of two fast phases of saccadic type with or without intersaccadic interval, respectively.
Cerebellum
December 2024
Faculty of Medicine, University of Zurich, Zurich, Switzerland.
Disease-specific oculomotor assessments play a crucial role in the early diagnosis of hereditary cerebellar ataxias. Whereas several studies have reported on quantitative oculomotor and vestibular measurements in Friedreich's Ataxia (FRDA), the value of specific oculomotor paradigms remains unclear. We aimed to address this knowledge gap through a systematic literature review and providing disease-specific recommendations for a tailored set of eye-movement recordings in FRDA.
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