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Caudal expiratory neuron in the monkey brainstem
138
Abstracts
Objective Multiple system atrophy (MSA) and pure autonomic failure (PAF) present mainly as symptoms of autonomic insufficiency, such as orthostatic hypotension and postprandial hypotension. We investigated postprandial hypotension in patients with MSA or PAF, and compared the severity between the two groups. Methods The investigation involved 10 patients with MSA (5 men, mean age 64 ± 9 years) and 4 patients with PAF (all men, mean age 73 ± 14 years). Each patient remained in the supine position throughout the meal-tolerance study, and blood pressure (BP) was intermittently measured from at least 15 min before to 60 min after the intake of an oral liquid diet (375 kcal), using automated sphygmomanometry. In addition, we performed a 70° head-up tilt test, and calculated the coefficient of R–R interval variability (CVR-R). Results There was no significant difference in the baseline systolic (S)/diastolic (D) BP between MSA (139 ± 12/75 ± 11) and PAF (131 ± 25/70 ± 16) patients. The preprandial S/DBP mean was 143 ± 21/78 ± 11 mm Hg in MSA patients and 149 ± 23/79 ± 10 mm Hg in PAF patients, with no significant difference between the 2 groups. Postprandial hypotension (SBP fall N 20 mm Hg) was observed in 7 of the MSA patients and in 1 of the PAF patients. PAF patients showed a greater S/ DBP fall after the meal (45 ± 22/19 ± 14 mm Hg) compared with MSA patients (28 ± 13/15 ± 13 mm Hg), although the difference between the two groups was not statistically significant. The lowest systolic blood pressure was observed 40 ± 10 min after the meal in MSA patients, and 46 ± 9 min in PAF patients. As for the results of the head-up tilt test, there was no significant difference in the S/DBP fall between MSA (41 ± 21 mm Hg) and PAF (44 ± 10 mm Hg) patients. Moreover, there was no significant difference in CVR-R values between MSA (1.38 ± 0.42%) and PAF (1.37 ± 0.31%) patients.
I-P-128 Autonomic and peripheral neuropathy in chromosome 16q22.1-linked autosomal dominant cerebellar ataxia Yoshiko Furiya a , Makito Hirano a , Masami Nomura b , Hirohide Asai a, Takao Kiriyama a, Satoshi Ueno a a Department of Neurology, Nara Medical University, Japan b Department of Neurology, Kainan Hospital Aichi Prefectural Welfare Federation of Agricultural Cooperatives, Japan A new type of ADCA, named chromosome 16q2.1-linked autosomal dominant cerebellar ataxia (16q-ADCA), was recently reported to be caused by a heterozygous C-T substitution in the 5′ non-coding region of puratrophin-1 gene. We searched for this mutation in168 patients from 129 families with ADCA and found it in 6 patients; they all showed late-onset pure cerebellar ataxia, but two had mild axonal neuropathy and orthostatic hypotension (OH). The results of nerve conduction studies revealed reduced compound muscle action potentials with slightly or mildly decreased nerve conduction velocities, indicating axonal sensorimotor neuropathy. On head-up-tilt test, increased plasma arginine-vasopressin concentrations and renin activity suggested that their OH was not due to involvement of the central autonomic nervous system. In one of two patients, abnormal QTc intervals and dispersion suggested involvement of the cardiac post-ganglionic sympathetic nervous system, which was supported by the abnormal findings of 123 I-MIBG-scintigraphy. Our findings are inconsistent with those previously reported in 16q-ADCA, but agree with those found in many other types of ADCA. Collectively, although only small numbers of patients have been studied available evidence suggests that 16q-ADCA may also involve nervous systems other than the cerebellum. doi:10.1016/j.autneu.2007.06.242
I-P-129 Caudal expiratory neuron in the monkey brainstem
Conclusion The postprandial BP fall was more pronounced in PAF patients than in MSA patients, although the results of the head-up tilt test showed no significant difference between the two groups. A difference in the severity of postprandial hypotension may reflect the underlying condition, being central and pre-ganglionic in MSA and post-ganglionic in PAF.
doi:10.1016/j.autneu.2007.06.241
Shuichi Sasaki a, Eiichi Oguni b, Sei-Ichi Sasaki c a Grad. Sch. Kansei Behavioral and Brain Sciences, Univ Tsukuba, Ibaraki, Japan b Ibaraki Pref. Central Hosp, Ibaraki, Japan c Ibaraki Pref. University of Helth. Sci., Centr. for Med. Sci., Ibaraki, Japan The basic breathing rhythm of higher mammals takes place in the medulla. Most studies have been made on the cat, rabbit and rat. However, little is known about the location and firing patterns of medullary respiratory neurons
Abstracts
in the monkey. This study was carried out to investigate the location of respiratory neurons in the monkey brainstem caudal to the obex. Experiment was performed on 1 adult monkey, anesthetized with sodium pentobarbital. Animal was paralyzed and kept on artificial ventilation. The phrenic nerve was dissected free, ligated, and cut distally. The brainstem was exposed by a caudal craniotomy. The spinal cord was exposed by a laminectomy at the Sl–S3 spinal levels. Glass micropipettes filled with 2 M NaCl solution saturated with Fast green FCF dye were used for extracellular recordings of single respiratory neurons. Spinal projection of respiratory neurons was tested by monopolar stimulation through a small Ag/AgCl ball electrode placed on the surface of the S2 spinal cord. Conduction velocities of descending stem axons were measured from the length of the spinal cord and the values of antidromic latencies. A total of 12 extracellular recordings were made. These were expiratory (E) neurons (12/12). Eleven of 12 E neurons were augmenting type and 1 neuron was unclassified. Ten of 12 neurons extended their descending axons in S2 spinal level. All spinal axons descended in the contralateral spinal cord with respect to the cell body of E neurons. Mean conduction velocities were 24.7 ± 4.3 m/s. Recording sites were found in the nucleus retroambigualis, 3.5 ± 0.8 mm caudal from the obex, 3.2 ± 0.5 mm ventral from the dorsal surface and 2.7 ± 0.3 mm lateral from the midline. The evidence in the present study suggests that the function of E neurons in the caudal nucleus retroambigualis is unlikely to be one of only respiration through intercostal muscles. E neurons might be involved in the important role of co-ordinating respiration and motor functions in the lumbar and the sacral spinal cord.
doi:10.1016/j.autneu.2007.06.243
I-P-130 A case of Guillain–Barre syndrome presenting severe blood pressure fluctuations
Mana Higashihara a,b, Masahiro Sonoo c, Satoko Unno b,d, Hideji Hashida b, Katsuhiko Takeda b,d a Department of Neurology, Division of Neuroscience, Graduate School of Medicine, the University of Tokyo, Japan b Department of Neurology, Japanese Red Cross Medical Center, Japan c Department of Neurology, Teikyo University, Japan d Department of Neurology, International University of Health and Welfare Mita Hospital, Japan
Blood pressure fluctuations due to the autonomic disturbances in Guillain–Barre syndrome (GBS) have been
139
described in previous reports and may develop to a lifethreatening condition. We here report a GBS patient who presented severe blood pressure fluctuations and was successfully treated with intravenous immunoglobulin. A 75-year-old woman was admitted to the emergency department of our hospital for rapidly evolving weakness, preceded ten days by a respiratory infection. She had a history of hypertension with systolic blood pressure (SBP) of around 140 mm Hg, but her SBP on admission was above 200 mm Hg. Heart rate was normal. She also presented respiratory failure due to respiratory muscle paresis. Other general findings included pneumonia in the right lower lobe of the lung and hypoactive bowel movement. Neurological examinations revealed quadriplegia with areflexia and dysphagia. Sensory symptoms or signs were not evident. CSF cells and total protein were not elevated. Nerve conduction studies performed on the next day showed decreased amplitudes of compound muscle action potentials in the median, ulnar and tibial nerves, normal conduction velocities, slightly prolonged distal latency in the median nerve, and conduction block in the ulnar nerve, generally indicating an axonal type of GBS. Anti-GD1b and anti-GM1 antibodies were later found positive in her serum. Based on these findings, we made a diagnosis of GBS with dysautonomia. Intravenous nicardipine hydrochloride was immediately started for the high blood pressure. As soon as we performed the intratracheal intubation using 10 mg intravenous propofol, her SBP suddenly dropped to 40 mm Hg. With rapid administration of dopamine and noradrenaline, the hypotension recovered, but her SBP varied from 80 mm Hg to 200 mm Hg in minutes thereafter while intravenous dopamine and propofol were continuously delivered. Because of the unstable hemodynamics, we judged that the plasma exchange had a higher risk, and therefore chose IVIg for treatment. On the first day of admission, the highest SBP was 238 mm Hg and the lowest SBP was 60 mm Hg, hence the greatest value of variation within a day in SBP was 178 mm Hg. Day by day, the peak in the SBP fell and the bottom in the SBP rose, and therefore the maximum value of variation in SBP decreased, and reached plateau on the fifth day. Her muscle power started to recover from the second day of admission. IVIg seems to be a treatment of choice for the GBS showing severe blood pressure fluctuations, which is safely administered and is and is effective not only for motor paralysis but for dysatuonomia.
doi:10.1016/j.autneu.2007.06.244
I-P-131 Neurotrimin is an estrogen-inducible, uterine protein that mediates sympathetic axon repulsion
Abstracts
Objective Multiple system atrophy (MSA) and pure autonomic failure (PAF) present mainly as symptoms of autonomic insufficiency, such as orthostatic hypotension and postprandial hypotension. We investigated postprandial hypotension in patients with MSA or PAF, and compared the severity between the two groups. Methods The investigation involved 10 patients with MSA (5 men, mean age 64 ± 9 years) and 4 patients with PAF (all men, mean age 73 ± 14 years). Each patient remained in the supine position throughout the meal-tolerance study, and blood pressure (BP) was intermittently measured from at least 15 min before to 60 min after the intake of an oral liquid diet (375 kcal), using automated sphygmomanometry. In addition, we performed a 70° head-up tilt test, and calculated the coefficient of R–R interval variability (CVR-R). Results There was no significant difference in the baseline systolic (S)/diastolic (D) BP between MSA (139 ± 12/75 ± 11) and PAF (131 ± 25/70 ± 16) patients. The preprandial S/DBP mean was 143 ± 21/78 ± 11 mm Hg in MSA patients and 149 ± 23/79 ± 10 mm Hg in PAF patients, with no significant difference between the 2 groups. Postprandial hypotension (SBP fall N 20 mm Hg) was observed in 7 of the MSA patients and in 1 of the PAF patients. PAF patients showed a greater S/ DBP fall after the meal (45 ± 22/19 ± 14 mm Hg) compared with MSA patients (28 ± 13/15 ± 13 mm Hg), although the difference between the two groups was not statistically significant. The lowest systolic blood pressure was observed 40 ± 10 min after the meal in MSA patients, and 46 ± 9 min in PAF patients. As for the results of the head-up tilt test, there was no significant difference in the S/DBP fall between MSA (41 ± 21 mm Hg) and PAF (44 ± 10 mm Hg) patients. Moreover, there was no significant difference in CVR-R values between MSA (1.38 ± 0.42%) and PAF (1.37 ± 0.31%) patients.
I-P-128 Autonomic and peripheral neuropathy in chromosome 16q22.1-linked autosomal dominant cerebellar ataxia Yoshiko Furiya a , Makito Hirano a , Masami Nomura b , Hirohide Asai a, Takao Kiriyama a, Satoshi Ueno a a Department of Neurology, Nara Medical University, Japan b Department of Neurology, Kainan Hospital Aichi Prefectural Welfare Federation of Agricultural Cooperatives, Japan A new type of ADCA, named chromosome 16q2.1-linked autosomal dominant cerebellar ataxia (16q-ADCA), was recently reported to be caused by a heterozygous C-T substitution in the 5′ non-coding region of puratrophin-1 gene. We searched for this mutation in168 patients from 129 families with ADCA and found it in 6 patients; they all showed late-onset pure cerebellar ataxia, but two had mild axonal neuropathy and orthostatic hypotension (OH). The results of nerve conduction studies revealed reduced compound muscle action potentials with slightly or mildly decreased nerve conduction velocities, indicating axonal sensorimotor neuropathy. On head-up-tilt test, increased plasma arginine-vasopressin concentrations and renin activity suggested that their OH was not due to involvement of the central autonomic nervous system. In one of two patients, abnormal QTc intervals and dispersion suggested involvement of the cardiac post-ganglionic sympathetic nervous system, which was supported by the abnormal findings of 123 I-MIBG-scintigraphy. Our findings are inconsistent with those previously reported in 16q-ADCA, but agree with those found in many other types of ADCA. Collectively, although only small numbers of patients have been studied available evidence suggests that 16q-ADCA may also involve nervous systems other than the cerebellum. doi:10.1016/j.autneu.2007.06.242
I-P-129 Caudal expiratory neuron in the monkey brainstem
Conclusion The postprandial BP fall was more pronounced in PAF patients than in MSA patients, although the results of the head-up tilt test showed no significant difference between the two groups. A difference in the severity of postprandial hypotension may reflect the underlying condition, being central and pre-ganglionic in MSA and post-ganglionic in PAF.
doi:10.1016/j.autneu.2007.06.241
Shuichi Sasaki a, Eiichi Oguni b, Sei-Ichi Sasaki c a Grad. Sch. Kansei Behavioral and Brain Sciences, Univ Tsukuba, Ibaraki, Japan b Ibaraki Pref. Central Hosp, Ibaraki, Japan c Ibaraki Pref. University of Helth. Sci., Centr. for Med. Sci., Ibaraki, Japan The basic breathing rhythm of higher mammals takes place in the medulla. Most studies have been made on the cat, rabbit and rat. However, little is known about the location and firing patterns of medullary respiratory neurons
Abstracts
in the monkey. This study was carried out to investigate the location of respiratory neurons in the monkey brainstem caudal to the obex. Experiment was performed on 1 adult monkey, anesthetized with sodium pentobarbital. Animal was paralyzed and kept on artificial ventilation. The phrenic nerve was dissected free, ligated, and cut distally. The brainstem was exposed by a caudal craniotomy. The spinal cord was exposed by a laminectomy at the Sl–S3 spinal levels. Glass micropipettes filled with 2 M NaCl solution saturated with Fast green FCF dye were used for extracellular recordings of single respiratory neurons. Spinal projection of respiratory neurons was tested by monopolar stimulation through a small Ag/AgCl ball electrode placed on the surface of the S2 spinal cord. Conduction velocities of descending stem axons were measured from the length of the spinal cord and the values of antidromic latencies. A total of 12 extracellular recordings were made. These were expiratory (E) neurons (12/12). Eleven of 12 E neurons were augmenting type and 1 neuron was unclassified. Ten of 12 neurons extended their descending axons in S2 spinal level. All spinal axons descended in the contralateral spinal cord with respect to the cell body of E neurons. Mean conduction velocities were 24.7 ± 4.3 m/s. Recording sites were found in the nucleus retroambigualis, 3.5 ± 0.8 mm caudal from the obex, 3.2 ± 0.5 mm ventral from the dorsal surface and 2.7 ± 0.3 mm lateral from the midline. The evidence in the present study suggests that the function of E neurons in the caudal nucleus retroambigualis is unlikely to be one of only respiration through intercostal muscles. E neurons might be involved in the important role of co-ordinating respiration and motor functions in the lumbar and the sacral spinal cord.
doi:10.1016/j.autneu.2007.06.243
I-P-130 A case of Guillain–Barre syndrome presenting severe blood pressure fluctuations
Mana Higashihara a,b, Masahiro Sonoo c, Satoko Unno b,d, Hideji Hashida b, Katsuhiko Takeda b,d a Department of Neurology, Division of Neuroscience, Graduate School of Medicine, the University of Tokyo, Japan b Department of Neurology, Japanese Red Cross Medical Center, Japan c Department of Neurology, Teikyo University, Japan d Department of Neurology, International University of Health and Welfare Mita Hospital, Japan
Blood pressure fluctuations due to the autonomic disturbances in Guillain–Barre syndrome (GBS) have been
139
described in previous reports and may develop to a lifethreatening condition. We here report a GBS patient who presented severe blood pressure fluctuations and was successfully treated with intravenous immunoglobulin. A 75-year-old woman was admitted to the emergency department of our hospital for rapidly evolving weakness, preceded ten days by a respiratory infection. She had a history of hypertension with systolic blood pressure (SBP) of around 140 mm Hg, but her SBP on admission was above 200 mm Hg. Heart rate was normal. She also presented respiratory failure due to respiratory muscle paresis. Other general findings included pneumonia in the right lower lobe of the lung and hypoactive bowel movement. Neurological examinations revealed quadriplegia with areflexia and dysphagia. Sensory symptoms or signs were not evident. CSF cells and total protein were not elevated. Nerve conduction studies performed on the next day showed decreased amplitudes of compound muscle action potentials in the median, ulnar and tibial nerves, normal conduction velocities, slightly prolonged distal latency in the median nerve, and conduction block in the ulnar nerve, generally indicating an axonal type of GBS. Anti-GD1b and anti-GM1 antibodies were later found positive in her serum. Based on these findings, we made a diagnosis of GBS with dysautonomia. Intravenous nicardipine hydrochloride was immediately started for the high blood pressure. As soon as we performed the intratracheal intubation using 10 mg intravenous propofol, her SBP suddenly dropped to 40 mm Hg. With rapid administration of dopamine and noradrenaline, the hypotension recovered, but her SBP varied from 80 mm Hg to 200 mm Hg in minutes thereafter while intravenous dopamine and propofol were continuously delivered. Because of the unstable hemodynamics, we judged that the plasma exchange had a higher risk, and therefore chose IVIg for treatment. On the first day of admission, the highest SBP was 238 mm Hg and the lowest SBP was 60 mm Hg, hence the greatest value of variation within a day in SBP was 178 mm Hg. Day by day, the peak in the SBP fell and the bottom in the SBP rose, and therefore the maximum value of variation in SBP decreased, and reached plateau on the fifth day. Her muscle power started to recover from the second day of admission. IVIg seems to be a treatment of choice for the GBS showing severe blood pressure fluctuations, which is safely administered and is and is effective not only for motor paralysis but for dysatuonomia.
doi:10.1016/j.autneu.2007.06.244
I-P-131 Neurotrimin is an estrogen-inducible, uterine protein that mediates sympathetic axon repulsion