Canadian Medical Guide > Diseases > Nervous System Diseases > Nervous System Malformations Terms and Definitions

Nervous System Malformations

Medical Definition:	Structural abnormalities of the central or peripheral nervous system resulting primarily from defects of embryogenesis.
Guide Notes:	for congen struct abnorm only; GEN or unspecified; prefer specifics or specific organ/abnorm; DF: NERVOUS SYSTEM ABNORM
Also Called:	Cranioschisis
Previously Indexed:	Nervous System/abnormalities (1966-1997)

Nervous System Malformations Categories.

	Central Nervous System Vascular Malformations - Congenital or acquired malformations involving arteries, veins, or venous sinuses of the brain, spinal cord, and meninges.
	Hereditary Motor and Sensory Neuropathies - A group of slowly progressive inherited disorders affecting motor and sensory peripheral nerves. Subtypes include HMSNs I-VII. HMSN I and II both refer to CHARCOT-MARIE-TOOTH DISEASE. HMSN III refers to hypertrophic neuropathy of infancy, HMSN IV refers to REFSUM DISEASE, HMSN V refers to a condition marked by a hereditary motor and sensory neuropathy associated with spastic paraplegia (see SPASTIC PARAPLEGIA, HEREDITARY). HMSN VI refers to HMSN associated with an inherited optic atrophy (OPTIC ATROPHIES, HEREDITARY), and HMSN VII refers to HMSN associated with retinitis pigmentosa. (From Adams et al., Principles of Neurology, 6th ed, p1343)
	Hydranencephaly - A congenital condition where the greater portions of the cerebral hemispheres and CORPUS STRIATUM are replaced by CSF and glial tissue. The meninges and the skull are well formed, which is consistent with earlier normal embryogenesis of the telencephalon. Bilateral occlusions of the internal carotid arteries in utero is a potential mechanism. Clinical features include intact brainstem reflexes without evidence of higher cortical activity. (Menkes, Textbook of Child Neurology, 5th ed, p307)
	Neural Tube Defects - Congenital malformations of the central nervous system and adjacent structures related to defective neural tube closure during the first trimester of pregnancy generally occurring between days 18-29 of gestation. Ectodermal and mesodermal malformations (mainly involving the skull and vertebrae) may occur as a result of defects of neural tube closure. (From Joynt, Clinical Neurology, 1992, Ch55, pp31-41)
	Septo-Optic Dysplasia - A condition resulting from congenital malformations involving the brain. The syndrome of septo-optic dysplasia combines hypoplasia or agenesis of the SEPTUM PELLUCIDUM and the OPTIC NERVE. The extent of the abnormalities can vary. Septo-optic dysplasia is often associated with abnormalities of the hypothalamic and other diencephalic structures, and HYPOPITUITARISM.

Nervous System Malformations Medical Definitions and Terms

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