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Strumpell's pure spastic paraplegia (SPSP): An electrophysiological demonstration of selective central distal axonopathy
Sl91
P34.05 CENTRAL AND PROXIMAL PERIPHERAL MOTOR C O N D U C T I O N IN A CASE OF C O M B I N E D MULTIPLE SCLEROSIS AND DEMYELINATING PERIPHERAL NEUROPATHY.
association with prolonged central conduction time (N13 N20). No clear correlations between the abnormalities demonstrated by neurophysiological investigations and the severity and duration of the illness were observed.
N.M.F Murray and K.R. Mills P34.07 (London, UK) Percutaneous stimulation of the brain and spinal cord was used to study pyramidal tract and peripheral motor conduction in a man with multiple sclerosis and demyelinating peripheral neuropathy. A low-output impedance stimulator was used to deliver brief shocks, up to 700 volts, 0.05-0.1 msec time-constant of decay, via surface electrodes. Surface recordings were made from Abductor Digiti Minimi during stimulation of the ulnar nerve at wrist, elbow and axilla, the cervical cord and the contralateral motor cortex. Cortex-cord conduction time was prolonged at 11.2 msec (normal: 4.4msec; SD 0.75). Motor conduction was slowed in proximal and distal segments of the peripheral nerve: Conduction velocity: cervical cord-axilla, 38 m/sec; axilla-elbow 28.8m/see; elbow-wrist, 29m/sec. With surface electrodes on Tibialis Anterior stimuli were applied to the peroneal nerve, the cauda equina (L4) and the spinal cord (L1,D1 and C6). Severe slowing of pyramidal tract conduction was demonstrated in the cord (C6-DI: 4.4m/see; DI-L1 4.0m/see), in the cauda equina (LI-L4:11.5m/see) and in the peripheral nerve (L4-Fibular head: 26.7m/sec). There was no response to stimulation of the leg area of the motor cortex.
The technique is of value in the assessment and localisation of lesions of central and peripheral motor pathways.
P34.06 AUDITORY AND S O M A T O S E N S O R Y EVOKED POTENTIALS IN OLIVOPONTOCEREBELLAR ATROPHY (OPCA) PATIENTS.
STRUMPELL'S
PURE
SPASTIC
PARAPLEGIA
(SPSP): AN ELECTROPHYSIOLOGICAL DEMONSTRAT I O N OF SELECTIVE CENTRAL DISTAL AXONOPATHY.
A. Uncmi, D. Gambi, M. Treviso and M. Basciani (Chieti, Italy) SPSP is an hereditary disorder characterized by progressive weakness, spasticity and impairment of deep sensation, predominantly of the lower limbs. The pathological alterations are: corticospinal tract degeneration from the medullary pyramids downwards increasing caudally, posterior column degeneration increasing rostrally, with intact posterior root fibres and peripheral nerves. These findings suggest a pattern of nervous system degeneration termed central distal axonopathy. We studied three subjects from the same family with an autosomal dominant SPSP, by electrophysiological techniques. Motor and sensory conduction velocities were determined in upper and lower limbs. Spinal and cortical SEPs by stimulation of median nerves, lumbosacral responses and SEPs by stimulation of peroneal nerves were also recorded. Peripheral nerve conduction, lumbosacral and Erb's point responses, spinal and cortical SEPs from median nerves were normal in all patients but cortical evoked potentials from peroneal stimulation were delayed and abnormal in wave-form, confirming a selective involvement of centripetal axons of primary sensory neurones, especially of the longest fibres running in the gracite fasciculi.
P34.08 S O M A T O S E N S O R Y EVOKED POTENTIALS IN C H R O N I C HUMAN VITAMIN E DEFICIENCY.
L. Ross~, G. De Scisciolo, S. Costantini, A. Bindi and R. Zappoli
S. Satya-Murti, L. Howard and S. Crane
(Florence, Italy)
(Albany, NY, USA)
Nine patients 4 with dominant and 5 with recessive a n d / o r sporadic OPCA were studied. All patients were submitted to SEP and AEP recordings, CT scan, vestibular and E M G - E N G examinations. AEP recordings included BAEPs and long latency components (LLCs). Peripheral and central components of SEPs were obtained by stimulation of median nerve at the wrist. No clear BAEP abnormality was observed, although a slight reduction of the I V - V / I amplitude ratio was found in 5 patients. Three of them were affected by dominant OPCA. LLCs (N85) were normal in all patients but one. SEP abnormalities were found in 5 patients and it is noteworthy that 4 (,f them were the patients affected by dominant OPCA. The main feature of SEP abnormalities was a delayed N20 in
Chronic vitamin E deficiency produces a syndrome resembling spinocerebellar degeneration. Somatosensory pathways are involved clinically and pathologically (Lancet 1983, 1; 225: J. Neuropathol. Exp. Neurol, 1981, 40; 166). However, studies of evoked potentials in this condition are not readily available. Therefore, we performed somatosensory EPs (SEPs) in 11 patients with chronic, long-standing vitamin E deficiency. The median or posterior tibial nerve was stimulated unilaterally. Erb's point (E), 7th cervical vertebra (C7), and contralateral parietal (P) electrode placements referenced to Fpz, were used for upper extremity stimulation. First lumbar vertebral to iliac crest (LI-IC) and Cz' to Fpz placements were used for lower extremity stimulation. Our subjects had vitamin
P34.05 CENTRAL AND PROXIMAL PERIPHERAL MOTOR C O N D U C T I O N IN A CASE OF C O M B I N E D MULTIPLE SCLEROSIS AND DEMYELINATING PERIPHERAL NEUROPATHY.
association with prolonged central conduction time (N13 N20). No clear correlations between the abnormalities demonstrated by neurophysiological investigations and the severity and duration of the illness were observed.
N.M.F Murray and K.R. Mills P34.07 (London, UK) Percutaneous stimulation of the brain and spinal cord was used to study pyramidal tract and peripheral motor conduction in a man with multiple sclerosis and demyelinating peripheral neuropathy. A low-output impedance stimulator was used to deliver brief shocks, up to 700 volts, 0.05-0.1 msec time-constant of decay, via surface electrodes. Surface recordings were made from Abductor Digiti Minimi during stimulation of the ulnar nerve at wrist, elbow and axilla, the cervical cord and the contralateral motor cortex. Cortex-cord conduction time was prolonged at 11.2 msec (normal: 4.4msec; SD 0.75). Motor conduction was slowed in proximal and distal segments of the peripheral nerve: Conduction velocity: cervical cord-axilla, 38 m/sec; axilla-elbow 28.8m/see; elbow-wrist, 29m/sec. With surface electrodes on Tibialis Anterior stimuli were applied to the peroneal nerve, the cauda equina (L4) and the spinal cord (L1,D1 and C6). Severe slowing of pyramidal tract conduction was demonstrated in the cord (C6-DI: 4.4m/see; DI-L1 4.0m/see), in the cauda equina (LI-L4:11.5m/see) and in the peripheral nerve (L4-Fibular head: 26.7m/sec). There was no response to stimulation of the leg area of the motor cortex.
The technique is of value in the assessment and localisation of lesions of central and peripheral motor pathways.
P34.06 AUDITORY AND S O M A T O S E N S O R Y EVOKED POTENTIALS IN OLIVOPONTOCEREBELLAR ATROPHY (OPCA) PATIENTS.
STRUMPELL'S
PURE
SPASTIC
PARAPLEGIA
(SPSP): AN ELECTROPHYSIOLOGICAL DEMONSTRAT I O N OF SELECTIVE CENTRAL DISTAL AXONOPATHY.
A. Uncmi, D. Gambi, M. Treviso and M. Basciani (Chieti, Italy) SPSP is an hereditary disorder characterized by progressive weakness, spasticity and impairment of deep sensation, predominantly of the lower limbs. The pathological alterations are: corticospinal tract degeneration from the medullary pyramids downwards increasing caudally, posterior column degeneration increasing rostrally, with intact posterior root fibres and peripheral nerves. These findings suggest a pattern of nervous system degeneration termed central distal axonopathy. We studied three subjects from the same family with an autosomal dominant SPSP, by electrophysiological techniques. Motor and sensory conduction velocities were determined in upper and lower limbs. Spinal and cortical SEPs by stimulation of median nerves, lumbosacral responses and SEPs by stimulation of peroneal nerves were also recorded. Peripheral nerve conduction, lumbosacral and Erb's point responses, spinal and cortical SEPs from median nerves were normal in all patients but cortical evoked potentials from peroneal stimulation were delayed and abnormal in wave-form, confirming a selective involvement of centripetal axons of primary sensory neurones, especially of the longest fibres running in the gracite fasciculi.
P34.08 S O M A T O S E N S O R Y EVOKED POTENTIALS IN C H R O N I C HUMAN VITAMIN E DEFICIENCY.
L. Ross~, G. De Scisciolo, S. Costantini, A. Bindi and R. Zappoli
S. Satya-Murti, L. Howard and S. Crane
(Florence, Italy)
(Albany, NY, USA)
Nine patients 4 with dominant and 5 with recessive a n d / o r sporadic OPCA were studied. All patients were submitted to SEP and AEP recordings, CT scan, vestibular and E M G - E N G examinations. AEP recordings included BAEPs and long latency components (LLCs). Peripheral and central components of SEPs were obtained by stimulation of median nerve at the wrist. No clear BAEP abnormality was observed, although a slight reduction of the I V - V / I amplitude ratio was found in 5 patients. Three of them were affected by dominant OPCA. LLCs (N85) were normal in all patients but one. SEP abnormalities were found in 5 patients and it is noteworthy that 4 (,f them were the patients affected by dominant OPCA. The main feature of SEP abnormalities was a delayed N20 in
Chronic vitamin E deficiency produces a syndrome resembling spinocerebellar degeneration. Somatosensory pathways are involved clinically and pathologically (Lancet 1983, 1; 225: J. Neuropathol. Exp. Neurol, 1981, 40; 166). However, studies of evoked potentials in this condition are not readily available. Therefore, we performed somatosensory EPs (SEPs) in 11 patients with chronic, long-standing vitamin E deficiency. The median or posterior tibial nerve was stimulated unilaterally. Erb's point (E), 7th cervical vertebra (C7), and contralateral parietal (P) electrode placements referenced to Fpz, were used for upper extremity stimulation. First lumbar vertebral to iliac crest (LI-IC) and Cz' to Fpz placements were used for lower extremity stimulation. Our subjects had vitamin