Upward Gaze Paralysis as the Initial Sign of Fisher's Syndrome

Keane, James R.; Finstead, Bruce A.

doi:10.1001/archneur.1982.00510240043012

Cited by 24 publications

(6 citation statements)

References 5 publications

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“…However, the sensory ataxia occasionally seen in GBS is quite different from that of SOAA (1 1). Eight of our patients, as well as 16 cases reported in the literature, complained of vertigo (2,4,5,(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23). Ataxic dysarthria was noted in 6 of our patients, and was mentioned in 27 cases in the literature (2, 5 , 6, 8, 20, 24-42).…”

Section: Discussionmentioning

confidence: 50%

Ataxia and areflexia in SOAA

Aldin

Trontelj

Anderson

et al. 1993

Acta Neurologica Scandinavica

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Fifteen patients with the classical syndrome of ophthalmoplegia, ataxia, and tendon areflexia (SOAA) were studied in an attempt to clarify the mechanisms of ataxia and myotatic hyporeflexia. All showed features of cerebellar rather than sensory ataxia. Peripheral nerve conduction studies, including F-waves, were normal in a majority of the patients, as was needle EMG. Low-amplitude compound sensory nerve potentials were seen in four patients only, and mild slowing of sensory conduction velocity in two. Three had abnormal blink reflex studies, suggestive of a central lesion in two, and another two showed a transient delay of N5 peak of brainstem auditory evoked potentials. Somatosensory evoked potentials were normal. Despite clinically depressed or absent tendon jerks, T-waves were elicited at normal latencies. These findings do not support the prevailing view that the neurological abnormalities in SOAA are due to involvement of sensory fibres in the peripheral nerves and dorsal roots. We suggest that lesions scattered in the brainstem tegmentum and in the cerebellar peduncles are responsible for the ataxia and the depressed tendon jerks.

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Section: Discussionmentioning

confidence: 50%

Ataxia and areflexia in SOAA

Aldin

Trontelj

Anderson

et al. 1993

Acta Neurologica Scandinavica

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“…l , 2). Bell's phenomenon was studied in 13 patients (3,(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) and was noted in the acute phase of illness despite paralysis of upgaze in 6 cases (8-1 1, 14, 15). Doll's head maneuver was tested for in cases 1, 3, 6-17 and it was found to increase the range of movement of both eyes in 4 (Cases 8, 9, 1 1, 15).…”

Section: Resultsmentioning

confidence: 99%

“…Since the original description of the syndrome, there is no consensus of opinion as to the site of lesion. Several cases have been reported in whom I the oculomotor signs indicated a brain stem disorder (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24). In a prospective study of the neuroophthalmic manifestations of SOAA we report our findings in 20 classical cases and argue that the site of the lesion is central rather than peripheral.…”

mentioning

confidence: 99%

Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia and areflexia Observations on 20 patients

Aldin

Anderson²,

Eeg‐Olofsson

et al. 2009

Acta Neurologica Scandinavica

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The neuro‐ophthalmological manifestations of 20 patients with the syndrome of ophthalmoplegia, ataxia and areflexia are described. The symmetrical nature of the ophthalmoplegia and the associated cerebellar ataxia point to centrally placed lesions. Several supranuclear, nuclear and internuclear ophthalmological signs are identified. Some of these, like partial sparing of the levator palpebrae and normal downgaze in the presence of severe ophthalmoplegia are noted too frequently to be just unusual signs of peripheral oculomotor dysfunction. Other identified features included upper lid retraction on attempted upgaze and preserved Bell's phenomenon in the presence of paralysis of the latter, as well as several other central ophthalmological signs. These findings contrast with those seen in the Guillain‐Barré syndrome and, thus, the syndrome of ophthalmoplegia, ataxia and areflexia is not a mere variant of it.

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“…Paediatric upgaze palsy as observed in our cohort is in general a rare clinical sign. Other paediatric neurological contexts in which it has been reported include hydrocephalus,7 Parinaud syndrome,7 the Miller-Fisher variant of Guillain-Barre syndrome,8 vitamin B12 deficiency,9 and Niemann-Pick disease type C 10…”

Section: Discussionmentioning

confidence: 99%

Ophthalmic features ofPLA2G6-related paediatric neurodegeneration with brain iron accumulation

et al. 2014

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Upgaze palsy, although not a previously reported finding, was confirmed in all patients (except in one for whom assessment could not be performed) and thus can be considered part of the phenotype in children and young adults. Other frequent findings not previously highlighted were abnormal convergence, saccadic pursuit, and saccadic intrusions. Optic nerve head pallor and strabismus, previously reported findings in the disease, were found in 100% and 50% of our cohort, respectively, and the strabismus in our series was always exotropia. Taken together, these clinical findings may be helpful in distinguishing PLA2G6-related neurodegeneration from the other major cause of NBIA, recessive PANK2 mutations.

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Upward Gaze Paralysis as the Initial Sign of Fisher's Syndrome

Cited by 24 publications

References 5 publications

Ataxia and areflexia in SOAA

Ataxia and areflexia in SOAA

Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia and areflexia Observations on 20 patients

Ophthalmic features ofPLA2G6-related paediatric neurodegeneration with brain iron accumulation

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