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Alcoholic neuropathy-clinical cases with prevailing impairment of the autonomic nervous system
Session 21: Peripheral Neuropathy I predominant root involvement (persisting SLR, absent ‘F’response) 10. and ii) those with predominant nerve involvement (absent SLR, delayed but
F-wave was recorded from abductor poilicis brevis (R) by stimulating median nerve at elbow supramaximally Latency of F-wave was measured.
preserved ‘F’response) 8, (iii) significant root and nerve involvement. Only 3 of 10 in group (i) improved as compared to 6 of the 8 in group (ii),
F-wave conduction velocity (FWCV) was then calculated and F-ratio was derived to compare the conduction time in distal and proximal segments of median nerve. Neurophysiologic data were collected 6 figures beyond the range of control mean Z?3 S.D. were taken as abnormal. While analyzing different parameters, Latency of H-refiex & HWCV were abnormal in 4% (p < 0.001; highly significant) tt 5/25 (200/o) patients (p < 0.01; significant) respectively. Latency of F-wave and FWCV were abnormal in 12125 (48%) Et 2125 (8%) patients (p < 0.001; highly significant) respectively. F-ratio was abnormal in 1125 (4%) patients (p < 0.02; not significant). Based on above observations it was found that abnormalities in late responses (H-reflex & F-wave) do occur even in latent phase of IAP Hence it can be said that subclinical neuropathy does occur in asymptomatic phase of IAP
and 1 of 3 of group (iii). CiDP is a neuropethy with diverse clinical associations. It is suggested that early as weil predominant evidence of root involvement may be one markerfor a bad prognosis.
12147
! ~~~g~~~tal Axonal Neuropathy
E. Lalumiere, M. Vanasse, J, Michaud. HopifalSfe-Justine Enfant, LKversite de Montreal, Montreal, Canada
and Hopifal Marie
Congenital neuropathy with prevailing axonai changes is an extremely rare entity since to our knowledge only one case has been reported up to now [l]. Over the last seven years, we have seen seven patients affected by such a neuropathy. A,ll patients had nerve conduction velocities suggestive of axonal involvement. in five children, the diagnosis was further documented by sural nerve biopsies (3) or post mortem studies (2) which showed primary axonal changes with secondary myelin degeneration. In three children, symptoms were already severe at birth and two of them (two brothers) died at 23 days and three months of age respectively. The five surviving patients had severe psychomotor retardation. in all patients, brain CT Scan showed cerebral atrophy. Extensive metaboiic investigation was normal in the five children in whom it was done. [I]
Guzzetta F., Ferriere G. Congenital neuropathy changes. Acta Neuropathol. 1985.68: 185-l 90.
Incidence and Features of Critical 21 08 l_--.-l--l p~iyne~r~pat~ies in Mechanically
with
prevailing
axonal
Illness Associated Ventilated Patients
F. Leijten. A.W. De Weerd, J.E. Harinck-De Weerd. Westeinde Hospital, The Hagwe, The Netherlands Critically ill polyneuropathy (CIP) is defined as a predominantly motor, axonal polynewropathy of unknown etiology, with acute onset after or during development of respiratory insufficiency in the context of sepsis or multiple organ dysfunction syndrome (MODS). We tested the hypothesis that CIP may be an early feature of this syndrome and studied its electromyographic course. At our general intensive care unit, we prospectively followed a cohort of 70 patients, who were under 75 years of age and on the respirator for five days. Each three days their clinical condition was scored on a polyneuropathy rating scale. EMGs were performed on day 5-7 and two weeks later. Electromyographic evidence for CIP was found in 35% of cases, other abnormalities in IrY’10 and inconclusive results in 21%. There was a clear correlation with degree of MODS. Mortality was 40%. In survivors, mobilization was protracted in those with EMG abnormalities, though prognosis was favorable after 1 year in most cases. Spontaneous muscle fiber activitycouid be found as early as 5 days after onset of critical illness. CIP has a high prevalence in those ventilated for more than 5 days, is a major cause for prolonged revalidation and has characteristic electromyographic features.
/ 21-09
1 Late Responses (H-Reflex & F-Wave) in Latent l~t~rrn~~e~t Acute-Porphyria (IAP)
R.K. Makkar, A. Poonia. D.K. Kochar. S.F! Medical India
College, Bikaner 334002
Late responses (H-reflex b F-wave) are invaluable neurophysiologic investigations as they may be abnormal even when conventional nerve conduction velocities (NCVs) are normal. Hitherto, very few studies have been performed in latent stage of Ikl? Hence, this study was carried out in 25 asymptomatic patients of IAP whose clinical neurological examination was completely normal. A group of 15 age b sex matched normal healthy persons served as control. H-reflex was recorded fram Gastrocnemius-soleus (R) by stimulating tibia1 nerve submaximally at popliteal fossa. Latency of H-reflex was measured, H-wave conduction velocity (HWCV) was then calculated as a mixed (motor-sensory) velocity.
21-I0 Sensory-Motor Recklinghausen
Polyneur~~at~~ Neurofi~r~mat~s~a
L. Pasqui, M. Castagna, R. Amerio, 0. Garbin, A. Simonatr *, N. Rrzzuio *. Division of Neurology - Este, Padova; “lnstitcite of Neurology:University of Verona Von Recklinghausen Neurofibromatosis (NF-1) is a genetic disorder transmitted as an autosomic dominant trait. The gene responsible for the disease has been mapped on chromosome 17 (17q11.2). Main signs and symptoms (cafe-aulait macules, axillary and inguinai freckling, iris hamartomas. neurofibromas) develop from cells derived from the neural crest. Rarely NF-1 has been associated with polyneuropathy. Neuropathological findings are reported of sural nerve biopsies of 2 NF-1 patients who had both clinical symptoms and neurophysiological features of axonal, progressive sensory-motor polyneuropathy. In both cases nerve fascicles were unequaily affected: some of theme were completely normal, whereas in others endoneurial structures were focally replaced by elongated cells within collagen fibrils. Ultrastructurally, elongated ceils were encircled by basa! membrane. These cells were immunostained byschwann cell markers. Progressive, focal replacement of nerve fascicles by neurofibroma-like structures may justifythe clinical course of the disease in the described patients. Such circumscribed pattern of growth, however, is suggestive for to be a self-limiting process, affecting the majority of nerve trunks, but different from what it is commonly observed in neurofibromas.
121-I
1 1 Alcoholic Neuropathy-Clinical Impairment of the Autonomic
Beatrice Popescu, Anca Popescu. Depaflment University Hospital, Bucharest, Romania
aus bastes ofNeuroiogx
Clinical
The authors present ihree clinical cases of alcoholic neuropathy in which the main symptoms purport the impairment of the autonomic nervous system. A 59-year old patient was admitted to our hospital for an enhanced postural discomfort The orthostatic blood pressure sagged to 50 mmHg (systolic pressure), while the pulse mounted to 120 per minute. Neurologically, the patient elicited a pyramidal and extrapyramidal symptomatology, with a Parkinsonian aspect. Tendon reflexes were abclished distally in all four limbs. The level of plasma cathecolamines was diminished (especially that of epinephrine). Clinical symptoms improved notabiy after several months of abstinence from alcoholic drinks. Dystrophies were noticed in two other patients with alcoholic neuropathy, who used to ingest large amounts of spirits. These patients developed erythema and bullae and later on, an necrotic ulceration affecting the toes (although the peripheral circulation was permeable and the patients did not suffer from diabetes mellitus) without complaining of serious pain. However, they presented an important impairment of pain sensitivity in the lower limbs, distally. This symptomatology suggests a somatoautonomic neuropathy, substantiated by the orthostatic hypotension and ceaseless tachycardia. Morphopathologic alterations in autonomic Bangiia during chronic alcoholism are known and recorded.
F-wave was recorded from abductor poilicis brevis (R) by stimulating median nerve at elbow supramaximally Latency of F-wave was measured.
preserved ‘F’response) 8, (iii) significant root and nerve involvement. Only 3 of 10 in group (i) improved as compared to 6 of the 8 in group (ii),
F-wave conduction velocity (FWCV) was then calculated and F-ratio was derived to compare the conduction time in distal and proximal segments of median nerve. Neurophysiologic data were collected 6 figures beyond the range of control mean Z?3 S.D. were taken as abnormal. While analyzing different parameters, Latency of H-refiex & HWCV were abnormal in 4% (p < 0.001; highly significant) tt 5/25 (200/o) patients (p < 0.01; significant) respectively. Latency of F-wave and FWCV were abnormal in 12125 (48%) Et 2125 (8%) patients (p < 0.001; highly significant) respectively. F-ratio was abnormal in 1125 (4%) patients (p < 0.02; not significant). Based on above observations it was found that abnormalities in late responses (H-reflex & F-wave) do occur even in latent phase of IAP Hence it can be said that subclinical neuropathy does occur in asymptomatic phase of IAP
and 1 of 3 of group (iii). CiDP is a neuropethy with diverse clinical associations. It is suggested that early as weil predominant evidence of root involvement may be one markerfor a bad prognosis.
12147
! ~~~g~~~tal Axonal Neuropathy
E. Lalumiere, M. Vanasse, J, Michaud. HopifalSfe-Justine Enfant, LKversite de Montreal, Montreal, Canada
and Hopifal Marie
Congenital neuropathy with prevailing axonai changes is an extremely rare entity since to our knowledge only one case has been reported up to now [l]. Over the last seven years, we have seen seven patients affected by such a neuropathy. A,ll patients had nerve conduction velocities suggestive of axonal involvement. in five children, the diagnosis was further documented by sural nerve biopsies (3) or post mortem studies (2) which showed primary axonal changes with secondary myelin degeneration. In three children, symptoms were already severe at birth and two of them (two brothers) died at 23 days and three months of age respectively. The five surviving patients had severe psychomotor retardation. in all patients, brain CT Scan showed cerebral atrophy. Extensive metaboiic investigation was normal in the five children in whom it was done. [I]
Guzzetta F., Ferriere G. Congenital neuropathy changes. Acta Neuropathol. 1985.68: 185-l 90.
Incidence and Features of Critical 21 08 l_--.-l--l p~iyne~r~pat~ies in Mechanically
with
prevailing
axonal
Illness Associated Ventilated Patients
F. Leijten. A.W. De Weerd, J.E. Harinck-De Weerd. Westeinde Hospital, The Hagwe, The Netherlands Critically ill polyneuropathy (CIP) is defined as a predominantly motor, axonal polynewropathy of unknown etiology, with acute onset after or during development of respiratory insufficiency in the context of sepsis or multiple organ dysfunction syndrome (MODS). We tested the hypothesis that CIP may be an early feature of this syndrome and studied its electromyographic course. At our general intensive care unit, we prospectively followed a cohort of 70 patients, who were under 75 years of age and on the respirator for five days. Each three days their clinical condition was scored on a polyneuropathy rating scale. EMGs were performed on day 5-7 and two weeks later. Electromyographic evidence for CIP was found in 35% of cases, other abnormalities in IrY’10 and inconclusive results in 21%. There was a clear correlation with degree of MODS. Mortality was 40%. In survivors, mobilization was protracted in those with EMG abnormalities, though prognosis was favorable after 1 year in most cases. Spontaneous muscle fiber activitycouid be found as early as 5 days after onset of critical illness. CIP has a high prevalence in those ventilated for more than 5 days, is a major cause for prolonged revalidation and has characteristic electromyographic features.
/ 21-09
1 Late Responses (H-Reflex & F-Wave) in Latent l~t~rrn~~e~t Acute-Porphyria (IAP)
R.K. Makkar, A. Poonia. D.K. Kochar. S.F! Medical India
College, Bikaner 334002
Late responses (H-reflex b F-wave) are invaluable neurophysiologic investigations as they may be abnormal even when conventional nerve conduction velocities (NCVs) are normal. Hitherto, very few studies have been performed in latent stage of Ikl? Hence, this study was carried out in 25 asymptomatic patients of IAP whose clinical neurological examination was completely normal. A group of 15 age b sex matched normal healthy persons served as control. H-reflex was recorded fram Gastrocnemius-soleus (R) by stimulating tibia1 nerve submaximally at popliteal fossa. Latency of H-reflex was measured, H-wave conduction velocity (HWCV) was then calculated as a mixed (motor-sensory) velocity.
21-I0 Sensory-Motor Recklinghausen
Polyneur~~at~~ Neurofi~r~mat~s~a
L. Pasqui, M. Castagna, R. Amerio, 0. Garbin, A. Simonatr *, N. Rrzzuio *. Division of Neurology - Este, Padova; “lnstitcite of Neurology:University of Verona Von Recklinghausen Neurofibromatosis (NF-1) is a genetic disorder transmitted as an autosomic dominant trait. The gene responsible for the disease has been mapped on chromosome 17 (17q11.2). Main signs and symptoms (cafe-aulait macules, axillary and inguinai freckling, iris hamartomas. neurofibromas) develop from cells derived from the neural crest. Rarely NF-1 has been associated with polyneuropathy. Neuropathological findings are reported of sural nerve biopsies of 2 NF-1 patients who had both clinical symptoms and neurophysiological features of axonal, progressive sensory-motor polyneuropathy. In both cases nerve fascicles were unequaily affected: some of theme were completely normal, whereas in others endoneurial structures were focally replaced by elongated cells within collagen fibrils. Ultrastructurally, elongated ceils were encircled by basa! membrane. These cells were immunostained byschwann cell markers. Progressive, focal replacement of nerve fascicles by neurofibroma-like structures may justifythe clinical course of the disease in the described patients. Such circumscribed pattern of growth, however, is suggestive for to be a self-limiting process, affecting the majority of nerve trunks, but different from what it is commonly observed in neurofibromas.
121-I
1 1 Alcoholic Neuropathy-Clinical Impairment of the Autonomic
Beatrice Popescu, Anca Popescu. Depaflment University Hospital, Bucharest, Romania
aus bastes ofNeuroiogx
Clinical
The authors present ihree clinical cases of alcoholic neuropathy in which the main symptoms purport the impairment of the autonomic nervous system. A 59-year old patient was admitted to our hospital for an enhanced postural discomfort The orthostatic blood pressure sagged to 50 mmHg (systolic pressure), while the pulse mounted to 120 per minute. Neurologically, the patient elicited a pyramidal and extrapyramidal symptomatology, with a Parkinsonian aspect. Tendon reflexes were abclished distally in all four limbs. The level of plasma cathecolamines was diminished (especially that of epinephrine). Clinical symptoms improved notabiy after several months of abstinence from alcoholic drinks. Dystrophies were noticed in two other patients with alcoholic neuropathy, who used to ingest large amounts of spirits. These patients developed erythema and bullae and later on, an necrotic ulceration affecting the toes (although the peripheral circulation was permeable and the patients did not suffer from diabetes mellitus) without complaining of serious pain. However, they presented an important impairment of pain sensitivity in the lower limbs, distally. This symptomatology suggests a somatoautonomic neuropathy, substantiated by the orthostatic hypotension and ceaseless tachycardia. Morphopathologic alterations in autonomic Bangiia during chronic alcoholism are known and recorded.