Biomaterial Database

Cochlear nerve degeneration coincident with adrenocerebroleukodystrophy. 1976

M Igarashi, and J G Neely, and P F Anthony, and B R Alford

Department of Otorhinolaryngology and Communicative Sciences, Baylor College of Medicine, Houston, TX 77030.

A 19-month-old boy had adrenocerebroleukodystrophy and unusually severe cochlear nerve degeneration and demyelination, a condition in advance of that expected from known disease entities. We report the only known temporal bone study in adrenocerebroleukodystrophy.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D002549	Diffuse Cerebral Sclerosis of Schilder	A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73)	Alpers Syndrome,Balo Concentric Sclerosis,Cerebral Sclerosis, Diffuse,Encephalitis Periaxialis,Myelinoclastic Diffuse Sclerosis,Poliodystrophia Cerebri,Schilder Disease,Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis,Alpers Disease,Alpers Progressive Infantile Poliodystrophy,Alpers' Disease,Alpers' Syndrome,Alpers-Huttenlocher Syndrome,Balo's Concentric Sclerosis,Encephalitis Periaxialis Concentrica,Encephalitis Periaxialis Diffusa,Neuronal Degeneration Of Childhood With Liver Disease, Progressive,Progressive Neuronal Degeneration of Childhood with Liver Disease,Progressive Sclerosing Poliodystrophy,Schilder's Disease,Sudanophilic Cerebral Sclerosis,Alper Disease,Alper Syndrome,Alper's Disease,Alper's Syndrome,Alpers Huttenlocher Syndrome,Concentric Sclerosis, Balo,Concentric Sclerosis, Balo's,Diffuse Cerebral Scleroses,Diffuse Cerebral Sclerosis,Diffuse Scleroses, Myelinoclastic,Diffuse Sclerosis, Myelinoclastic,Disease, Alpers',Disease, Schilder,Disease, Schilder's,Myelinoclastic Diffuse Scleroses,Progressive Sclerosing Poliodystrophies,Schilders Disease,Scleroses, Balo's Concentric,Scleroses, Myelinoclastic Diffuse,Sclerosis, Diffuse Cerebral,Sclerosis, Myelinoclastic Diffuse,Syndrome, Alpers,Syndrome, Alpers-Huttenlocher
D003056	Cochlear Nerve	The cochlear part of the 8th cranial nerve (VESTIBULOCOCHLEAR NERVE). The cochlear nerve fibers originate from neurons of the SPIRAL GANGLION and project peripherally to cochlear hair cells and centrally to the cochlear nuclei (COCHLEAR NUCLEUS) of the BRAIN STEM. They mediate the sense of hearing.	Acoustic Nerve,Auditory Nerve,Acoustic Nerves,Auditory Nerves,Cochlear Nerves,Nerve, Acoustic,Nerve, Auditory,Nerve, Cochlear,Nerves, Acoustic,Nerves, Auditory,Nerves, Cochlear
D003711	Demyelinating Diseases	Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.	Clinically Isolated CNS Demyelinating Syndrome,Clinically Isolated Syndrome, CNS Demyelinating,Demyelinating Disorders,Demyelination,Demyelinating Disease,Demyelinating Disorder,Demyelinations
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000160	Vestibulocochlear Nerve Diseases	Pathological processes of the VESTIBULOCOCHLEAR NERVE, including the branches of COCHLEAR NERVE and VESTIBULAR NERVE. Common examples are VESTIBULAR NEURITIS, cochlear neuritis, and ACOUSTIC NEUROMA. Clinical signs are varying degree of HEARING LOSS; VERTIGO; and TINNITUS.	Acoustic Nerve Diseases,Cochlear Nerve Diseases,Cranial Nerve VIII Diseases,Vestibular Nerve Diseases,Acoustic Nerve Disorders,Cochlear Nerve Disorders,Cochlear Neuritis,Cranial Nerve VIII Disorders,Eighth Cranial Nerve Diseases,Vestibular Nerve Disorders,Acoustic Nerve Disease,Acoustic Nerve Disorder,Cochlear Nerve Disease,Cochlear Nerve Disorder,Cochlear Neuritides,Neuritides, Cochlear,Neuritis, Cochlear,Vestibular Nerve Disease,Vestibular Nerve Disorder,Vestibulocochlear Nerve Disease
D000326	Adrenoleukodystrophy	An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).	Adrenomyeloneuropathy,Schilder-Addison Complex,X-Linked Adrenoleukodystrophy,ALD (Adrenoleukodystrophy),Addison Disease and Cerebral Sclerosis,Bronze Schilder Disease,Melanodermic Leukodystrophy,Siemerling-Creutzfeldt Disease,X-ALD,X-ALD (X-Linked Adrenoleukodystrophy),Adrenoleukodystrophy, X-Linked,Leukodystrophies, Melanodermic,Leukodystrophy, Melanodermic,Schilder Addison Complex,Siemerling Creutzfeldt Disease,X ALD,X ALD (X Linked Adrenoleukodystrophy),X Linked Adrenoleukodystrophy
D013701	Temporal Bone	Either of a pair of compound bones forming the lateral (left and right) surfaces and base of the skull which contains the organs of hearing. It is a large bone formed by the fusion of parts: the squamous (the flattened anterior-superior part), the tympanic (the curved anterior-inferior part), the mastoid (the irregular posterior portion), and the petrous (the part at the base of the skull).	Stylomastoid Foramen,Bone, Temporal,Temporal Bones