Periodic Alternating Nystagmus in Friedreich's Ataxia*

Gorman, Warren F.; Brock, Samuel

doi:10.1016/0002-9394(50)91599-6

Cited by 30 publications

(3 citation statements)

References 2 publications

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“…This sign is frequently described as of cerebellar origin (Zee et al, 1976). Also, there was no periodic alternating nystagmus as previously described by Gorman and Brock (1950). B) Calorics (Table 3) Most of the patients had normal calorics.…”

Section: Eng Studysupporting

confidence: 64%

Clinical and Electronystagmographic findings in Friedreich's Ataxia

Lemieux

et al. 1978

Can. j. neurol. sci.

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Section: Eng Studysupporting

confidence: 64%

Clinical and Electronystagmographic findings in Friedreich's Ataxia

Lemieux

et al. 1978

Can. j. neurol. sci.

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“…PAN is thought to arise from an unstable “velocity-storage mechanism”—a neural brainstem network that prolongs the duration of peripheral vestibular afferent signals from the semicircular canals ( 2 ). PAN is most often described in its infantile form ( 3 – 6 ), although acquired PAN has been associated with demyelinating disease ( 7 , 8 ), posterior fossa malformation ( 3 ), spinocerebellar degeneration ( 9 – 11 ), and anticonvulsant medications ( 12 , 13 ); in many cases the cause is never identified. There have been reported cases of PAN as a direct consequence of visual ( 14 ) or vestibular ( 15 ) loss, as a transient consequence of sensory deprivation ( 6 ).…”

Section: Introductionmentioning

confidence: 99%

Adult Periodic Alternating Nystagmus Masked by Involuntary Head Movements

et al. 2018

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Acquired periodic alternating nystagmus (PAN) describes a horizontal jerk nystagmus that reverses its direction with a predictable cycle, and is thought to arise from lesions involving the brainstem and cerebellum. We report a 20-year-old patient with PAN who presented with an acute vertiginous episode and developed an involuntary head movement that initially masked the PAN. The involuntary head movements were abolished with a subtherapeutic dose of botulinum toxin to the neck muscles. We propose that the head movements initially developed as a compensatory movement to the nystagmus, to maintain visual fixation in the presence of the underlying nystagmus, and became an entrained involuntary behavior. This case highlights the importance of disambiguating psychogenic from organic pathology as this may have clinical therapeutic implications, in this case resolution of the most disabling symptom which was her head oscillations, leading to improved day-to-day function despite PAN.

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“…Periodic alternating nystagmus (PAN) is a rare eye movement disorder that causes oscillopsia and degrades vision. It is associated with lesions of the vestibular nuclei and vestibulocerebellum [ [3, 181, spinocerebellar degeneration [8], and congenital nystagmus [ 3 , 4,6,141. In many cases the cause is never found [2,6].…”

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confidence: 99%