Somatosensory and brainstem auditory evoked potentials in neurodegenerative system disorders. 1987

P M Rossini, and J B Cracco

Brainstem auditory (BAEP) and somatosensory (SEP) evoked potentials to median and peroneal nerve stimulation were investigated in 25 patients with neurodegenerative system disorders: 9 Friedreich's ataxia, 7 hereditary motor sensory neuropathies, 3 familial spastic paraplegia, 3 olivopontocerebellar atrophy, 1 ataxia telangiectasia and 1 abetalipoproteinemia. BAEPs were abnormal in 39%, SEPs to both upper- and lower-limb stimulation were abnormal in 63%. Serial evoked potential testing paralleled the clinical progression. SEPs were more frequently and severely altered than BAEPs suggesting that SEP testing may be a more sensitive indicator of early involvement of afferent pathways in these disorders.

UI MeSH Term Description Entries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009422 Nervous System Diseases Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle. Neurologic Disorders,Nervous System Disorders,Neurological Disorders,Disease, Nervous System,Diseases, Nervous System,Disorder, Nervous System,Disorder, Neurologic,Disorder, Neurological,Disorders, Nervous System,Disorders, Neurologic,Disorders, Neurological,Nervous System Disease,Nervous System Disorder,Neurologic Disorder,Neurological Disorder
D009849 Olivopontocerebellar Atrophies A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS, PATHOLOGIC; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085) Dejerine-Thomas Syndrome,Olivopontocerebellar Atrophy, Idiopathic,Olivopontocerebellar Hypoplasia,Familial Olivopontocerebellar Atrophy,Inherited Olivopontocerebellar Atrophy,Nonfamilial Olivopontocerebellar Atrophy,Olivo-Ponto-Cerebellar Atrophy,Olivo-Ponto-Cerebellar Degeneration,Olivopontocerebellar Atrophy,Olivopontocerebellar Degeneration,Pontoolivocerebellar Atrophy,Presenile Ataxia,Ataxia, Presenile,Atrophy, Familial Olivopontocerebellar,Atrophy, Idiopathic Olivopontocerebellar,Atrophy, Inherited Olivopontocerebellar,Atrophy, Nonfamilial Olivopontocerebellar,Atrophy, Olivo-Ponto-Cerebellar,Atrophy, Olivopontocerebellar,Atrophy, Pontoolivocerebellar,Degeneration, Olivo-Ponto-Cerebellar,Degeneration, Olivopontocerebellar,Dejerine Thomas Syndrome,Familial Olivopontocerebellar Atrophies,Hypoplasia, Olivopontocerebellar,Idiopathic Olivopontocerebellar Atrophies,Idiopathic Olivopontocerebellar Atrophy,Inherited Olivopontocerebellar Atrophies,Nonfamilial Olivopontocerebellar Atrophies,Olivo Ponto Cerebellar Atrophy,Olivo Ponto Cerebellar Degeneration,Olivo-Ponto-Cerebellar Degenerations,Olivopontocerebellar Atrophies, Familial,Olivopontocerebellar Atrophies, Nonfamilial,Olivopontocerebellar Atrophy, Familial,Olivopontocerebellar Atrophy, Inherited,Olivopontocerebellar Atrophy, Nonfamilial,Olivopontocerebellar Degenerations,Olivopontocerebellar Hypoplasias,Pontoolivocerebellar Atrophies,Presenile Ataxias,Syndrome, Dejerine-Thomas
D010264 Paraplegia Severe or complete loss of motor function in the lower extremities and lower portions of the trunk. This condition is most often associated with SPINAL CORD DISEASES, although BRAIN DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; NEUROMUSCULAR DISEASES; and MUSCULAR DISEASES may also cause bilateral leg weakness. Paralysis, Lower Extremities,Paraplegia, Spastic,Spastic Paraplegia,Paralysis, Legs,Paralysis, Lower Limbs,Paraplegia, Ataxic,Paraplegia, Cerebral,Paraplegia, Flaccid,Paraplegia, Spinal,Ataxic Paraplegia,Ataxic Paraplegias,Cerebral Paraplegia,Cerebral Paraplegias,Flaccid Paraplegia,Flaccid Paraplegias,Paraplegias,Paraplegias, Ataxic,Paraplegias, Cerebral,Paraplegias, Flaccid,Paraplegias, Spastic,Paraplegias, Spinal,Spastic Paraplegias,Spinal Paraplegia,Spinal Paraplegias
D001933 Brain Stem The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA. Brainstem,Truncus Cerebri,Brain Stems,Brainstems,Cerebri, Truncus,Cerebrus, Truncus,Truncus Cerebrus
D002526 Cerebellar Diseases Diseases that affect the structure or function of the cerebellum. Cardinal manifestations of cerebellar dysfunction include dysmetria, GAIT ATAXIA, and MUSCLE HYPOTONIA. Cerebellar Dysfunction,Cerebellum Diseases,Cerebellar Disorders,Cerebellar Syndromes,Cerebellar Disease,Cerebellar Disorder,Cerebellar Dysfunctions,Cerebellar Syndrome,Cerebellum Disease,Disease, Cerebellar,Disease, Cerebellum,Disorder, Cerebellar,Dysfunction, Cerebellar,Syndrome, Cerebellar
D002607 Charcot-Marie-Tooth Disease A hereditary motor and sensory neuropathy transmitted most often as an autosomal dominant trait and characterized by progressive distal wasting and loss of reflexes in the muscles of the legs (and occasionally involving the arms). Onset is usually in the second to fourth decade of life. This condition has been divided into two subtypes, hereditary motor and sensory neuropathy (HMSN) types I and II. HMSN I is associated with abnormal nerve conduction velocities and nerve hypertrophy, features not seen in HMSN II. (Adams et al., Principles of Neurology, 6th ed, p1343) Atrophy, Muscular, Peroneal,HMSN Type I,HMSN Type II,Hereditary Motor and Sensory-Neuropathy Type II,Hereditary Motor, and Sensory Neuropathy Type I,Muscular Atrophy, Peroneal,Peroneal Muscular Atrophy,Roussy-Levy Syndrome,Charcot-Marie Disease,Charcot-Marie-Tooth Disease, Autosomal Dominant, With Focally Folded Myelin Sheaths, Type 1A,Charcot-Marie-Tooth Disease, Autosomal Dominant, with Focally Folded Myelin Sheaths, Type 1B,Charcot-Marie-Tooth Disease, Demyelinating, Type 1A,Charcot-Marie-Tooth Disease, Demyelinating, Type 1B,Charcot-Marie-Tooth Disease, Slow Nerve Conduction Type, Linked To Duffy,Charcot-Marie-Tooth Disease, Type 1A,Charcot-Marie-Tooth Disease, Type 1B,Charcot-Marie-Tooth Disease, Type I,Charcot-Marie-Tooth Disease, Type IA,Charcot-Marie-Tooth Disease, Type IB,Charcot-Marie-Tooth Disease, Type II,Charcot-Marie-Tooth Hereditary Neuropathy,Charcot-Marie-Tooth Neuropathy, Type 1A,Charcot-Marie-Tooth Neuropathy, Type 1B,Charcot-Marie-Tooth Syndrome,HMN Distal Type I,HMSN 1A,HMSN 1B,HMSN I,HMSN IA,HMSN IB,HMSN II,HMSN1A,HMSN1B,Hereditary Areflexic Dystasia,Hereditary Motor And Sensory Neuropathy IB,Hereditary Motor and Sensory Neuropathy 1A,Hereditary Motor and Sensory Neuropathy 1B,Hereditary Motor and Sensory Neuropathy IA,Hereditary Type I Motor and Sensory Neuropathy,Neuropathy, Type I Hereditary Motor and Sensory,Neuropathy, Type II Hereditary Motor and Sensory,Roussy Levy Hereditary Areflexic Dystasia,Roussy-Levy Disease,Roussy-Levy Hereditary Areflexic Dystasia,Areflexic Dystasia, Hereditary,Areflexic Dystasias, Hereditary,Atrophies, Peroneal Muscular,Atrophy, Peroneal Muscular,Charcot Marie Disease,Charcot Marie Tooth Disease,Charcot Marie Tooth Disease, Type 1A,Charcot Marie Tooth Disease, Type 1B,Charcot Marie Tooth Disease, Type I,Charcot Marie Tooth Disease, Type IA,Charcot Marie Tooth Disease, Type IB,Charcot Marie Tooth Disease, Type II,Charcot Marie Tooth Hereditary Neuropathy,Charcot Marie Tooth Neuropathy, Type 1A,Charcot Marie Tooth Neuropathy, Type 1B,Charcot Marie Tooth Syndrome,Dystasia, Hereditary Areflexic,Dystasias, Hereditary Areflexic,Hereditary Areflexic Dystasias,Hereditary Motor and Sensory Neuropathy Type II,Hereditary Neuropathy, Charcot-Marie-Tooth,Muscular Atrophies, Peroneal,Peroneal Muscular Atrophies,Roussy Levy Disease,Roussy Levy Syndrome,Syndrome, Charcot-Marie-Tooth,Syndrome, Roussy-Levy
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004568 Electrodiagnosis Diagnosis of disease states by recording the spontaneous electrical activity of tissues or organs or by the response to stimulation of electrically excitable tissue. Electrodiagnoses

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