Table 1: Presence of nystagmus in
Ophthalmologic syndromes and triads
Syndrome/Triad |
Incidence
of nystagmus |
Other
signs |
Upper
neuron disorders (+) |
Charcot’s
triad |
High |
Intention
tremor, nystagmus, staccato speech |
+ |
Peliazeus-Merzbacher
disease |
High |
|
+ |
Mitchap
60 disease |
High |
|
+ |
Metachromatic
leukodystrophy |
High |
|
+ |
Refsum
disease |
High |
|
+ |
Zellweger
disease |
High |
|
+ |
Alexander
disease |
High |
|
+ |
Canavan
disease |
High |
|
+ |
Krabbe'
disease |
High |
|
+ |
Vanishing
white matter |
High |
|
+ |
Aircadi-Goutie'res
syndrome |
High |
|
+ |
“Mais-
Nadim Nasser TRIAD” |
High |
Primary
hypotonia, nystagmus, abnormal BERA test |
+ |
Spasmus
nutans |
High |
Pendula
nystagmus, head nodding and torticollis. |
+ spontaneous
recovery after 1st year |
Optic
disc (foveal) hypoplasia. |
High |
nystagmus, |
Local
ophthalmic cause |
Gaucher
disease |
rare |
Trismus,
strabismus, and opisthotonus. |
+ Upper
neuron storage disease |
Kearns-Sayre
syndrome |
rare |
External
ophthalmo-plegia, pigmentary retinopathy, cardiac conduction block |
|
Congenital
glaucoma |
rare |
Epiphora,
blepharospasm and photophobia. |
|
Horner's
syndrome |
rare |
Ptosis,
miosis, ipsilateral anhidrosis of face |
|
Lambert–Eaton
myasthenic syndrome |
rare |
Muscle
hypotonia, autonomic dysfunction, hyporeflexia |
Neuromuscular
junction-Lower motor neuron |
Fechtner's
syndrome |
rare |
Nephritis,
sensorineural hearing loss, eye abnormalities |
|
Congenital
rubella retinopathy |
Low |
cataracts,
deafness, congenital heart disease |
|
This table shows the syndromes in pediatric
Ophthalmology, according to etiologies with high or low incidence of nystagmus,
and according to upper and lower neuron injuries.
It also
emphasizes that ICN is a major sign in all
myelin disorders, including Multiple
sclerosis, Paleazeus-Mertzbacher disease, Metachromatic leukodystropy and
etcetera.