Biomaterial Database

Peripheral and central somatosensory nerve conduction defects in Friedreich's ataxia. 1980

S J Jones, and M Baraitser, and A M Halliday

Somatosensory evoked potentials were recorded over the clavicle, cervical spine, mastoid processes and the hand area of the contralateral somatosensory cortex to median nerve stimulation in 22 cases of Friedreich's ataxia. There was a marked attenuation of the clavicular potential, but little evidence of delay of this or the major cervical component. A dispersed and delayed cortical response was therefore suggestive of slowed conduction in central pathways. The cortical response was shown to be mediated by peripheral fibres with normal conduction velocity between stimulation sites at the wrist and the elbow.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009431	Neural Conduction	The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.	Nerve Conduction,Conduction, Nerve,Conduction, Neural,Conductions, Nerve,Conductions, Neural,Nerve Conductions,Neural Conductions
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D005071	Evoked Potentials	Electrical responses recorded from nerve, muscle, SENSORY RECEPTOR, or area of the CENTRAL NERVOUS SYSTEM following stimulation. They range from less than a microvolt to several microvolts. The evoked potential can be auditory (EVOKED POTENTIALS, AUDITORY), somatosensory (EVOKED POTENTIALS, SOMATOSENSORY), visual (EVOKED POTENTIALS, VISUAL), or motor (EVOKED POTENTIALS, MOTOR), or other modalities that have been reported.	Event Related Potential,Event-Related Potentials,Evoked Potential,N100 Evoked Potential,P50 Evoked Potential,N1 Wave,N100 Evoked Potentials,N2 Wave,N200 Evoked Potentials,N3 Wave,N300 Evoked Potentials,N4 Wave,N400 Evoked Potentials,P2 Wave,P200 Evoked Potentials,P50 Evoked Potentials,P50 Wave,P600 Evoked Potentials,Potentials, Event-Related,Event Related Potentials,Event-Related Potential,Evoked Potential, N100,Evoked Potential, N200,Evoked Potential, N300,Evoked Potential, N400,Evoked Potential, P200,Evoked Potential, P50,Evoked Potential, P600,Evoked Potentials, N100,Evoked Potentials, N200,Evoked Potentials, N300,Evoked Potentials, N400,Evoked Potentials, P200,Evoked Potentials, P50,Evoked Potentials, P600,N1 Waves,N2 Waves,N200 Evoked Potential,N3 Waves,N300 Evoked Potential,N4 Waves,N400 Evoked Potential,P2 Waves,P200 Evoked Potential,P50 Waves,P600 Evoked Potential,Potential, Event Related,Potential, Event-Related,Potential, Evoked,Potentials, Event Related,Potentials, Evoked,Potentials, N400 Evoked,Related Potential, Event,Related Potentials, Event,Wave, N1,Wave, N2,Wave, N3,Wave, N4,Wave, P2,Wave, P50,Waves, N1,Waves, N2,Waves, N3,Waves, N4,Waves, P2,Waves, P50
D005260	Female		Females
D005621	Friedreich Ataxia	An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)	Friedreich Disease,Hereditary Spinal Sclerosis,Sclerosis, Hereditary Spinal,Friedreich Familial Ataxia,Friedreich Hereditary Ataxia,Friedreich Hereditary Spinal Ataxia,Friedreich Spinocerebellar Ataxia,Friedreich's Ataxia,Friedreich's Disease,Friedreich's Familial Ataxia,Friedreich's Hereditary Ataxia,Friedreich's Hereditary Spinal Ataxia,Hereditary Spinal Ataxia, Friedreich,Hereditary Spinal Ataxia, Friedreich's,Ataxia, Friedreich,Ataxia, Friedreich Familial,Ataxia, Friedreich Hereditary,Ataxia, Friedreich Spinocerebellar,Ataxia, Friedreich's,Ataxia, Friedreich's Familial,Ataxia, Friedreich's Hereditary,Ataxias, Friedreich,Ataxias, Friedreich's Hereditary,Disease, Friedreich,Disease, Friedreich's,Familial Ataxia, Friedreich,Familial Ataxia, Friedreich's,Friedreich Ataxias,Friedreich's Hereditary Ataxias,Friedreichs Familial Ataxia,Friedreichs Hereditary Ataxia,Hereditary Ataxia, Friedreich,Hereditary Ataxia, Friedreich's,Hereditary Ataxias, Friedreich's,Hereditary Spinal Scleroses,Scleroses, Hereditary Spinal,Spinal Scleroses, Hereditary,Spinal Sclerosis, Hereditary,Spinocerebellar Ataxia, Friedreich
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D013003	Somatosensory Cortex	Area of the parietal lobe concerned with receiving sensations such as movement, pain, pressure, position, temperature, touch, and vibration. It lies posterior to the central sulcus.	Brodmann Area 1,Brodmann Area 2,Brodmann Area 3,Brodmann Areas 1, 2, 3,Brodmann Areas 1, 2, and 3,Brodmann Areas 3, 1, 2,Brodmann Areas 3, 1, and 2,Brodmann's Area 1,Brodmann's Area 2,Brodmann's Area 3,Brodmann's Areas 1, 2, and 3,Brodmann's Areas 3, 1, and 2,Parietal-Opercular Cortex,Primary Somesthetic Area,S1 Cortex,S2 Cortex,SII Cortex,Anterior Parietal Cortex,Gyrus Postcentralis,Post Central Gyrus,Postcentral Gyrus,Primary Somatic Sensory Area,Primary Somatosensory Area,Primary Somatosensory Areas,Primary Somatosensory Cortex,SI Cortex,Second Somatic Sensory Area,Secondary Sensory Cortex,Secondary Somatosensory Area,Secondary Somatosensory Cortex,Area 1, Brodmann,Area 1, Brodmann's,Area 2, Brodmann,Area 2, Brodmann's,Area 3, Brodmann,Area 3, Brodmann's,Area, Primary Somatosensory,Area, Primary Somesthetic,Area, Secondary Somatosensory,Areas, Primary Somatosensory,Brodmanns Area 1,Brodmanns Area 2,Brodmanns Area 3,Cortex, Anterior Parietal,Cortex, Parietal-Opercular,Cortex, Primary Somatosensory,Cortex, S1,Cortex, S2,Cortex, SI,Cortex, SII,Cortex, Secondary Sensory,Cortex, Secondary Somatosensory,Cortex, Somatosensory,Gyrus, Post Central,Gyrus, Postcentral,Parietal Cortex, Anterior,Parietal Opercular Cortex,Parietal-Opercular Cortices,Primary Somatosensory Cortices,Primary Somesthetic Areas,S1 Cortices,S2 Cortices,SII Cortices,Secondary Somatosensory Areas,Sensory Cortex, Secondary,Somatosensory Area, Primary,Somatosensory Area, Secondary,Somatosensory Areas, Primary,Somatosensory Cortex, Primary,Somatosensory Cortex, Secondary,Somesthetic Area, Primary,Somesthetic Areas, Primary