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PS-54-11 ALS-like syndrome due to aluminium intoxication
Poster session 54. Motor neuron diseases showed delayed conduction velocities, and five patients among the eight had anti-glycolipid antibodies. In addition, two cases showed conduction block and one of these had high-titered monoclonal IgM anti-GM1 antibody. Our data failed to prove the idea that anti-glycolipid antibody might cause delayed conduction velocities in peripheral motor nerve, however, conduction block may be associated with high-titered anti-GM1 antibody.
P$-54-11
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ALS-like syndrome due to aluminium intoxication
TiJlin Tannda~,, Dilek Ince, Sibel Tekin, Karim Nourikhalichi, Sevin~ Aktan. Marmara University Hospital, Istanbul, Turkey Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of anterior horn cells. Wide range of etiological factors may cause ALS-like syndromes, such as heavy metal intoxication, endocrinopathies, paraproteinemias, paraneoplastic syndromes, multifocal motor neuropathies and cervical structural lesions. In this report, we present a case of 32 year-old woman with a history of progressive weakness in the extremities for two months. On neurological examination, she had weakness, atrophy and diffuse fasciculations in all extremities being marked in the arms. She had no sensory deficit. Routine screening tests were unremarkable. Electromyography ( E M G ) was consistent with diffuse, generalized axonopathy. In the muscles tested, there were abundant positive sharp waves, fibrillations and fasciculations. On voluntary contraction, there were rapid firing, long duration polyphasic potentials. Since she had been living in an area close to factories in an industrial city and showed a rapid clinical deterioration, we investigated toxicology. A m o n g heavy metals, aluminium level in serum (200 microg/L)) was markedly elevated compared to normal value of 1-14 microg/L. Aluminium level in the cerebrospinal fluid was 96 microg/l. All other risk factors were ruled out. Serum aluminium levels of her husband and three children were also elevated, but not as high as hers. They were normal electrophysiologically. As a treatment a chealating agent (desferoxamine 1000 rag/day) has been administered for four weeks. Control E M G showed signs of acute denervation and reinnervation with giant motor unit potentials in the paraspinal muscles and clinically her atrophy reversed to some extent. She had progressive decrease in serum aluminium level.
PS-54-12 ] Trial to communicate with amyotrophic lateral sclerosis (ALS) patients in totally locked-in state
$229
with the thumb and the ulnar fingers one by one; Internal Motor Sequence Test, simulate the same motor sequence internally; Continuous Contraction Test, sustained isometric contraction between the thumb and the little finger. During each paradigm, we recorded SEPs. Results: The frontal N30 component contralateral to the stimuli was depressed by the Continuous Contraction Test in comparison with those of the control. Other two conditions did not provoke changes in the amplitude of SEPs components.
Conclusion It seems to be difficult to use the complex tasks which require the planning of the motor-sequence test for communication with ALS patients in totally locked-in state.
[ PS-54-131 An uncommon presentation of fluorosis M.F. Khamees, M.K. Akbar Ali, M.I. Feroz Khan, Abdullah Eyadeh, V.D. Mohan, M.I. Izdeen, S.A Ul-haqe.
Physical Medicine & Rehabilitation Hospital, PO. Box 4079, Safat 13041, Kuwait A 70 years old farmer from Yemen was referred as a case of osteoarthritis of both knees for preoperative rehabilitation procedures. Six years before he developed progressive skeletal stiffness. By 70 years he became dependent for ambulation and many other self-care activities. He showed quadriparesis resulting from compression of spinal cord and nerve roots at multiple levels associated with multiple joint involvement. Electrodiagnosis: Needle E M G showed chronic neurogenic changes of motor unit potentials at different levels especially in the proximal muscles of the upper limbs. Evoked potential studies revealed absence or delayed SEPs on stimulation of both Median and Tibial nerves. Total myelography showed multiple indentation of the contrast column. Plain X-rays of pelvis, dorso-lumbar spines and the right forearm showed uniform increase in bone density with calcification of interosseous membrane and ligaments. Fluoride levels in the water sample from Yemen and the patient's urine were elevated. Apart from Macrocytic Anaemia, all other haematological and biochemical investigations were normal.Discussion: The fluoride level in the water sample from Yemen was relatively low when compared to the standards put down by the W H O and many other authors to cause a crippling skeletal fluorosis. The disability in this case could be due to drinking large amounts of cantaminated water for decades and working under the sun. Electrophysiological study was useful in confirming the diagnosis which showed features of upper and lower neurone lesion.
Shin-Ichiro Kajimoto l, Reiko Namba 2, Toshiyuki Hayabara 2.
1Department of neurology, Kenju Hospital, Japan; 2Department of Neurology, National Minami, Okayama Hospital, Japan Respiratory-supported ALS patients, resulting in totally locked-in state, cannot communicate with the environment via blinking or eyeball movement. It is known that the frontal N30 component of the somatosensory evoked potentials (SEPs) changes during planning and execution of finger movements. We tested this gating p h e n o m e n o n by which ALS patients should indicate "yes" or "no". Methods: SEPs were collected from normal volunteers. The electrodes were placed at frontal, central and parietal locations. Both ear served as a reference. Stimulus electrodes were placed on the median nerveat the wrist. The stimulus intensity was 3.5 times sensory threshold and 500 responses were averaged. The experiment began with a control recording during which no task being asked. Then, we tested the following paradigm: Motor Sequence Test, a quick sequence of opposing movements
[ PS-54-14 ] Repetitive motor unit firing in amyotrophic lateral sclerosis Andrew Eisen 1, Katarzyna Rowinska 2, lrena Hausmanowa-Petrusewicz 2. I University of British
Columbia, Vancouver, Canada; 2 Polish Academy of Sciences, Warszaw, Poland A motor unit is defined as firing repetitively when it discharges more than twice with an interspike interval half or less than the inherent firing frequency of the motor unit. Descriptions of repetitive discharges (Rds) are sparse and their origin and significance is obscure. We have observed that they are particularly evident in ALS when they are most readily recordable from the tibialis anterior muscle. RDs were sort for in 60 patients with ALS using a monpolar or concentric needle and a narrow bandpas (500 Hz-20 kHz).
P$-54-11
]
]
ALS-like syndrome due to aluminium intoxication
TiJlin Tannda~,, Dilek Ince, Sibel Tekin, Karim Nourikhalichi, Sevin~ Aktan. Marmara University Hospital, Istanbul, Turkey Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of anterior horn cells. Wide range of etiological factors may cause ALS-like syndromes, such as heavy metal intoxication, endocrinopathies, paraproteinemias, paraneoplastic syndromes, multifocal motor neuropathies and cervical structural lesions. In this report, we present a case of 32 year-old woman with a history of progressive weakness in the extremities for two months. On neurological examination, she had weakness, atrophy and diffuse fasciculations in all extremities being marked in the arms. She had no sensory deficit. Routine screening tests were unremarkable. Electromyography ( E M G ) was consistent with diffuse, generalized axonopathy. In the muscles tested, there were abundant positive sharp waves, fibrillations and fasciculations. On voluntary contraction, there were rapid firing, long duration polyphasic potentials. Since she had been living in an area close to factories in an industrial city and showed a rapid clinical deterioration, we investigated toxicology. A m o n g heavy metals, aluminium level in serum (200 microg/L)) was markedly elevated compared to normal value of 1-14 microg/L. Aluminium level in the cerebrospinal fluid was 96 microg/l. All other risk factors were ruled out. Serum aluminium levels of her husband and three children were also elevated, but not as high as hers. They were normal electrophysiologically. As a treatment a chealating agent (desferoxamine 1000 rag/day) has been administered for four weeks. Control E M G showed signs of acute denervation and reinnervation with giant motor unit potentials in the paraspinal muscles and clinically her atrophy reversed to some extent. She had progressive decrease in serum aluminium level.
PS-54-12 ] Trial to communicate with amyotrophic lateral sclerosis (ALS) patients in totally locked-in state
$229
with the thumb and the ulnar fingers one by one; Internal Motor Sequence Test, simulate the same motor sequence internally; Continuous Contraction Test, sustained isometric contraction between the thumb and the little finger. During each paradigm, we recorded SEPs. Results: The frontal N30 component contralateral to the stimuli was depressed by the Continuous Contraction Test in comparison with those of the control. Other two conditions did not provoke changes in the amplitude of SEPs components.
Conclusion It seems to be difficult to use the complex tasks which require the planning of the motor-sequence test for communication with ALS patients in totally locked-in state.
[ PS-54-131 An uncommon presentation of fluorosis M.F. Khamees, M.K. Akbar Ali, M.I. Feroz Khan, Abdullah Eyadeh, V.D. Mohan, M.I. Izdeen, S.A Ul-haqe.
Physical Medicine & Rehabilitation Hospital, PO. Box 4079, Safat 13041, Kuwait A 70 years old farmer from Yemen was referred as a case of osteoarthritis of both knees for preoperative rehabilitation procedures. Six years before he developed progressive skeletal stiffness. By 70 years he became dependent for ambulation and many other self-care activities. He showed quadriparesis resulting from compression of spinal cord and nerve roots at multiple levels associated with multiple joint involvement. Electrodiagnosis: Needle E M G showed chronic neurogenic changes of motor unit potentials at different levels especially in the proximal muscles of the upper limbs. Evoked potential studies revealed absence or delayed SEPs on stimulation of both Median and Tibial nerves. Total myelography showed multiple indentation of the contrast column. Plain X-rays of pelvis, dorso-lumbar spines and the right forearm showed uniform increase in bone density with calcification of interosseous membrane and ligaments. Fluoride levels in the water sample from Yemen and the patient's urine were elevated. Apart from Macrocytic Anaemia, all other haematological and biochemical investigations were normal.Discussion: The fluoride level in the water sample from Yemen was relatively low when compared to the standards put down by the W H O and many other authors to cause a crippling skeletal fluorosis. The disability in this case could be due to drinking large amounts of cantaminated water for decades and working under the sun. Electrophysiological study was useful in confirming the diagnosis which showed features of upper and lower neurone lesion.
Shin-Ichiro Kajimoto l, Reiko Namba 2, Toshiyuki Hayabara 2.
1Department of neurology, Kenju Hospital, Japan; 2Department of Neurology, National Minami, Okayama Hospital, Japan Respiratory-supported ALS patients, resulting in totally locked-in state, cannot communicate with the environment via blinking or eyeball movement. It is known that the frontal N30 component of the somatosensory evoked potentials (SEPs) changes during planning and execution of finger movements. We tested this gating p h e n o m e n o n by which ALS patients should indicate "yes" or "no". Methods: SEPs were collected from normal volunteers. The electrodes were placed at frontal, central and parietal locations. Both ear served as a reference. Stimulus electrodes were placed on the median nerveat the wrist. The stimulus intensity was 3.5 times sensory threshold and 500 responses were averaged. The experiment began with a control recording during which no task being asked. Then, we tested the following paradigm: Motor Sequence Test, a quick sequence of opposing movements
[ PS-54-14 ] Repetitive motor unit firing in amyotrophic lateral sclerosis Andrew Eisen 1, Katarzyna Rowinska 2, lrena Hausmanowa-Petrusewicz 2. I University of British
Columbia, Vancouver, Canada; 2 Polish Academy of Sciences, Warszaw, Poland A motor unit is defined as firing repetitively when it discharges more than twice with an interspike interval half or less than the inherent firing frequency of the motor unit. Descriptions of repetitive discharges (Rds) are sparse and their origin and significance is obscure. We have observed that they are particularly evident in ALS when they are most readily recordable from the tibialis anterior muscle. RDs were sort for in 60 patients with ALS using a monpolar or concentric needle and a narrow bandpas (500 Hz-20 kHz).