tuberous sclerosis

sclerosis /scle·ro·sis/ (-ro´sis) an induration or hardening, especially from inflammation and in diseases of the interstitial substance; applied chiefly to such hardening of the nervous system or to hardening of the blood vessels.

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amyotrophic lateral sclerosis  Lou Gehrig disease: progressive degeneration of the neurons that give rise to the corticospinal tract and of the motor cells of the brain stem and spinal cord, resulting in a deficit of upper and lower motor neurons; it usually has a fatal outcome within 2 to 3 years.

arterial sclerosis  arteriosclerosis.

arteriolar sclerosis  arteriolosclerosis.

diffuse cerebral sclerosis  the infantile form of metachromatic leukodystrophy.

disseminated sclerosis  multiple s.

familial centrolobar sclerosis  Pelizaeus-Merzbacher disease.

glomerular sclerosis  glomerulosclerosis.

hippocampal sclerosis  loss of neurons in the region of the hippocampus, with gliosis; sometimes seen in epilepsy.

lateral sclerosis  degeneration of the lateral columns of the spinal cord, leading to spastic paraplegia. See amyotrophic lateral sclerosis and primary lateral sclerosis

Mönckeberg's sclerosis  see under arteriosclerosis.

multiple sclerosis  (MS) demyelination occurring in patches throughout the white matter of the central nervous system, sometimes extending into the gray matter; symptoms of lesions of the white matter are weakness, incoordination, paresthesias, speech disturbances, and visual complaints.

primary lateral sclerosis  a form of motor neuron disease in which the degenerative process is limited to the corticospinal pathways.

progressive systemic sclerosis  systemic scleroderma.

tuberous sclerosis  an autosomal dominant disease characterized by hamartomas of the brain (tubers), retina, and viscera; mental retardation; seizures; and adenoma sebaceum.

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tuberous sclerosis

n.

An inherited disease characterized by hamartomas of the brain, retina, and viscera, as well as epileptic seizures, mental retardation, and skin nodules of the face. Also called Bourneville's disease.

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Tuberous sclerosis

A genetic disease that causes skin problems, seizures, and mental retardation. It may be confused with autism.

Mentioned in: Autism

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tuberous sclerosis,

a familial, neurocutaneous disease characterized by epilepsy, mental deterioration, adenoma sebaceum, nodules and sclerotic patches on the cerebral cortex, retinal tumors, depigmented leaf-shaped macules on the skin, tumors of the heart or kidneys, and cerebral calcifications. There is no effective treatment. Also called Bourneville's disease, epiloia. See also adenoma sebaceum.

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sclerosis [sklĕ-ro´sis]

an induration or hardening, especially of a part from inflammation, or in disease of the interstitial substance. The term is used chiefly for such a hardening of the nervous system due to hyperplasia of the connective tissue or for hardening of the blood vessels. Called also induration. adj., adj sclerot´ic.

amyotrophic lateral sclerosis see amyotrophic lateral sclerosis.

arteriolar sclerosis arteriolosclerosis.

disseminated sclerosis multiple sclerosis.

familial centrolobar sclerosis a progressive familial form of leukoencephalopathy marked by nystagmus, ataxia, tremor, parkinsonian facies, dysarthria, and mental deterioration.

focal glomerular sclerosis focal segmental glomerulosclerosis.

glomerular sclerosis glomerulosclerosis.

hippocampal sclerosis loss of neurons in the region of the hippocampus, with gliosis; sometimes seen in epilepsy.

lateral sclerosis a form seated in the lateral columns of the spinal cord. It may be primary, with spastic paraplegia, rigidity of the limbs, and increase of the tendon reflexes but no sensory disturbances, or secondary to myelitis, with paraplegia and sensory disturbance.

medial calcific sclerosis (Mönckeberg's sclerosis) Mönckeberg's arteriosclerosis.

multiple sclerosis see multiple sclerosis.

systemic sclerosis systemic scleroderma.

tuberous sclerosis a congenital heredofamilial disease, transmitted as an autosomal dominant trait, characterized principally by the presence of hamartomas of the brain (tubers), retina (phakomas), and viscera, mental retardation, seizures, and adenoma sebaceum, and often associated with other skin lesions.

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tuberous sclerosis

Internal medicine An AD neurocutaneous syndrome characterized by skin lesions, variable mental retardation and seizures; TS is named after the typical brain lesion–a tuber. See Neurocutaneous disorder.

Tuberous sclerosis –gomez criteria  

Primary^†  Secondary^‡  

Facial angiofibroma 1º relative with TS

Fibrous plaque on forehead Hypomelanic macules

Ungual fibroma  Shagreen patch

Cortical tuber Infantile spasms

Multiple retinal hamartomas Single retinal hamartoma

Subependymal hamartoma Cardiac rhabdomyoma

Bilateral renal angiomyolipomas or cysts

^†One required for diagnosis^‡Two required for diagnosis