Leigh's Subacute Necrotizing Encephalomyelopathy Manifesting as Spasmus Nutans

Sedwick, Lyn A.; Burde, Ronald M.; Hodges, Fred J.

doi:10.1001/archopht.1984.01040030848030

Cited by 25 publications

(3 citation statements)

References 14 publications

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“…Gliomas involving the optic nerves, chiasm, and the third ventricular region are the most common (Schulman et al 1979, Antony et al 1980, Albright et al 1984, Newman et al 1990). Asymmetric nystagmus has also been reported as the presenting sign of a thalamic lesion (Baram and Tang 1986), of an arachnoid cyst (Spaide et al 1985), of opsoclonus-myoclonus (Allarakhia and Trobe 1995), and of Leigh's subacute necrotizing encephalomyelopathy (Sedwick et al 1984).…”

mentioning

confidence: 99%

Diagnosing children presenting with asymmetric pendular nystagmus

Shawkat

Kriss

Russell‐Eggitt

et al. 2001

Develop Med Child Neuro

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Horizontal asymmetric nystagmus usually occurs in one of three situations: secondary to an intracranial lesion, with monocular visual loss, or as part of the triad that constitutes the diagnosis of spasmus nutans (asymmetric nystagmus, abnormal head posture, head shake). Clinical records of 277 children, presenting with congenital nystagmus over an 8-year period were reviewed. Nystagmus was asymmetric in 24 of 277 cases. Seven of these patients were diagnosed with spasmus nutans. This is a rare condition that is only diagnosed retrospectively based on the absence of any abnormal neuroimaging or electrophysiological findings. Twelve of 24 patients had intracranial pathology and all had abnormal visual evoked potentials (VEPs). Five patients were diagnosed with congenital sensory defect nystagmus including one with albinism, three with congenital cone dysfunction, and one with cone-rod dystrophy. This paper stresses that although neuroimaging is necessary in all patients presenting with asymmetric nystagmus, such nystagmus can also occur with retinal disease or albinism and indicates the importance of non-invasive VEP/ERG testing in all forms of nystagmus.

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mentioning

confidence: 99%

Diagnosing children presenting with asymmetric pendular nystagmus

Shawkat

Kriss

Russell‐Eggitt

et al. 2001

Develop Med Child Neuro

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show abstract

“…Other conditions found to be masquerading as SN include arachnoid cyst (the shunting of which can alleviate the nystagmus), 30 Leighs subacute necrotizing encephalomyopathy, 31 and a thalamic tumor. 27 Congenital stationary night blindness, 24,32 has presented with a SN-like presentation, so any child with SN and high myopia should undergo an ERG.…”

Section: Early Onset Nystagmusmentioning

confidence: 44%

Abnormal Eye Movements in Children

Dinkin¹,

Rizzo²

2008

International Ophthalmology Clinics

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“…Finally, there is a group of SN‐like conditions that reproduce the clinical picture of SN but are secondary. The causes of secondary SN‐like syndrome includes ophtalmological disorders (severe refractive errors, optic nerve and chiasma gliomas, and retinal disorders)71, 80, 83–85 and neurological disorders (vermian cerebellar hypoplasia,86 brain tumors,87–89 Pelizaeus Merzbacher disease, and Leigh's encephalomyelopathy) 90…”

Section: Introductionmentioning

confidence: 99%

Abstracts

2012

Head & Neck

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Leigh's Subacute Necrotizing Encephalomyelopathy Manifesting as Spasmus Nutans

Cited by 25 publications

References 14 publications

Diagnosing children presenting with asymmetric pendular nystagmus

Diagnosing children presenting with asymmetric pendular nystagmus

Abnormal Eye Movements in Children

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