Batten-mayou syndrome (spielmeyer-vogt syndrome; mayou-batten disease; stock-spielmeyer- vogt syndrome; cerebroretinal degeneration; pigmentary retinal lipoid neuronal heredodegeneration; vogt-spielmeyer syndrome; juvenile ganglioside lipidosis; neuronal  ceroid lipofuscinosis; myoclonic variant of cerebral lipidosis; batten disease; cerebromacular dystrophy; juvenile amaurotic family idiocy; spielmeyer-sjögren syndrome)   127

General: Autosomal recessive; some cases of autosomal dominant; possible disturbance in lipid metabolism; most common in Jewish families; onset between ages 5 and 8 years; mean age at death is 17 years; poor prognosis (see Tay-Sachs Disease; Dollinger-Bielschowsky Syndrome.) The lipopigment storage diseases are divided into four types based on clinical and electron microscopic features: infantile (Hagberg-Santavuori syndrome), late infantile (Jansky-Bielschowsky disease), juvenile (Spielmeyer- Vogt disease), and adult (Kufs disease).

Ocular: Vision initially reduced, progressing to total blindness; fat deposition in the retina with gradual development of pigment disturbances resembling retinitis pigmentosa; progressive primary optic atrophy; granular pigmentary change of macula; there is clinical evidence supporting the idea that the primary lesion of the retina is in the inner layers.

Clinical: Mental disturbances; convulsions (later); apathy; irritability; ataxia; upper and lower motor neuron palsies; rigidity; complete paralysis and dementia in terminal stage; hypertonus; death from intercurrent infection.

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