Evoked potentials in cerebrotendinous xanthomatosis and effect induced by chenodeoxycholic acid. 1992

M Mondelli, and A Rossi, and C Scarpini, and M T Dotti, and A Federico
Institute of Neurological Sciences, Medical School, University of Siena, Italy.

Evoked potentials are reported in 10 patients with cerebrotendinous xanthomatosis, eight of whom had peripheral neuropathy. Four subjects showed delayed N13 to N20 interpeak latencies for arm somatosensory evoked potentials, and five showed moderately prolonged I to III and I to V interpeak latencies of brain-stem auditory evoked potentials. Six of seven patients showed marked delay and desynchronization of visual evoked potentials. All five patients undergoing transcutaneous magnetic stimulation of the motor cortex presented greatly delayed central motor conduction time, especially of the lower limbs. After treatment with chenodiol (750 mg/d for at least 2 years), there was a significant improvement in nerve conduction velocities, N13 to N20 interpeak latencies, and visual evoked potential latencies. Brain-stem auditory evoked potentials remained unchanged.

UI MeSH Term Description Entries
D008280 Magnetics The study of MAGNETIC PHENOMENA. Magnetic
D008297 Male Males
D009044 Motor Cortex Area of the FRONTAL LOBE concerned with primary motor control located in the dorsal PRECENTRAL GYRUS immediately anterior to the central sulcus. It is comprised of three areas: the primary motor cortex located on the anterior paracentral lobule on the medial surface of the brain; the premotor cortex located anterior to the primary motor cortex; and the supplementary motor area located on the midline surface of the hemisphere anterior to the primary motor cortex. Brodmann Area 4,Brodmann Area 6,Brodmann's Area 4,Brodmann's Area 6,Premotor Cortex and Supplementary Motor Cortex,Premotor and Supplementary Motor Cortices,Anterior Central Gyrus,Gyrus Precentralis,Motor Area,Motor Strip,Precentral Gyrus,Precentral Motor Area,Precentral Motor Cortex,Premotor Area,Premotor Cortex,Primary Motor Area,Primary Motor Cortex,Secondary Motor Areas,Secondary Motor Cortex,Somatic Motor Areas,Somatomotor Areas,Supplementary Motor Area,Area 4, Brodmann,Area 4, Brodmann's,Area 6, Brodmann,Area 6, Brodmann's,Area, Motor,Area, Precentral Motor,Area, Premotor,Area, Primary Motor,Area, Secondary Motor,Area, Somatic Motor,Area, Somatomotor,Area, Supplementary Motor,Brodmann's Area 6s,Brodmanns Area 4,Brodmanns Area 6,Central Gyrus, Anterior,Cortex, Motor,Cortex, Precentral Motor,Cortex, Premotor,Cortex, Primary Motor,Cortex, Secondary Motor,Cortices, Secondary Motor,Gyrus, Anterior Central,Gyrus, Precentral,Motor Area, Precentral,Motor Area, Primary,Motor Area, Secondary,Motor Area, Somatic,Motor Areas,Motor Cortex, Precentral,Motor Cortex, Primary,Motor Cortex, Secondary,Motor Strips,Precentral Motor Areas,Precentral Motor Cortices,Premotor Areas,Primary Motor Areas,Primary Motor Cortices,Secondary Motor Area,Secondary Motor Cortices,Somatic Motor Area,Somatomotor Area,Supplementary Motor Areas
D010523 Peripheral Nervous System Diseases Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. Peripheral Nerve Diseases,Peripheral Neuropathies,PNS (Peripheral Nervous System) Diseases,PNS Diseases,Peripheral Nervous System Disease,Peripheral Nervous System Disorders,Nerve Disease, Peripheral,Nerve Diseases, Peripheral,Neuropathy, Peripheral,PNS Disease,Peripheral Nerve Disease,Peripheral Neuropathy
D001927 Brain Diseases Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM. Intracranial Central Nervous System Disorders,Brain Disorders,CNS Disorders, Intracranial,Central Nervous System Disorders, Intracranial,Central Nervous System Intracranial Disorders,Encephalon Diseases,Encephalopathy,Intracranial CNS Disorders,Brain Disease,Brain Disorder,CNS Disorder, Intracranial,Encephalon Disease,Encephalopathies,Intracranial CNS Disorder
D002635 Chenodeoxycholic Acid A bile acid, usually conjugated with either glycine or taurine. It acts as a detergent to solubilize fats for intestinal absorption and is reabsorbed by the small intestine. It is used as cholagogue, a choleretic laxative, and to prevent or dissolve gallstones. Chenic Acid,Chenodeoxycholate,Chenodiol,Gallodesoxycholic Acid,Chenique Acid,Chenix,Chenofalk,Chenophalk,Henohol,Quenobilan,Quenocol,Sodium Chenodeoxycholate,Acid, Chenic,Acid, Chenique,Acid, Chenodeoxycholic,Acid, Gallodesoxycholic,Chenodeoxycholate, Sodium
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
D005073 Evoked Potentials, Somatosensory The electric response evoked in the CEREBRAL CORTEX by stimulation along AFFERENT PATHWAYS from PERIPHERAL NERVES to CEREBRUM. Somatosensory Evoked Potentials,Evoked Potential, Somatosensory,Somatosensory Evoked Potential
D005074 Evoked Potentials, Visual The electric response evoked in the cerebral cortex by visual stimulation or stimulation of the visual pathways. Visual Evoked Response,Evoked Potential, Visual,Evoked Response, Visual,Evoked Responses, Visual,Potential, Visual Evoked,Potentials, Visual Evoked,Response, Visual Evoked,Responses, Visual Evoked,Visual Evoked Potential,Visual Evoked Potentials,Visual Evoked Responses
D005260 Female Females

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