Treatment of Orthostatic Hypotension in Familial Amyloid Polyneuropathy with L-Threo-3,4-Dihydroxyphenylserine

Suzuki, Tomokazu; Sakoda, Saburo; Higa, Sadayoshi; Ueji, Masao; Kishimoto, Susumu; Hayashi, Akira; Takaba, Yoshio; Nakajima, Akira

doi:10.1007/978-1-4613-2199-6_56

Cited by 3 publications

(6 citation statements)

References 3 publications

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“…Among these cases, Case 1 was the first in which all of the patient's symptoms could be attributed to OD. In addition, the patient's peripheral sympathetic nerve response based on variations in the plasma noradrenalin level during position changes was inadequate (31).…”

Section: Orthostatic Intolerancementioning

confidence: 99%

Peripheral Sympathetic Nerve Dysfunction in Adolescent Japanese Girls Following Immunization with the Human Papillomavirus Vaccine

et al. 2014

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Objective To investigate the causes of neurological manifestations in girls immunized with the human papillomavirus (HPV) vaccine. Methods During the past nine months, 44 girls visited us complaining of several symptoms after HPV vaccination. Four patients with other proven disorders were excluded, and the remaining forty subjects were enrolled in this study. Results The age at initial vaccination ranged from 11 to 17 years, and the average incubation period after the first dose of the vaccine was 5.47±5.00 months. Frequent manifestations included headaches, general fatigue, coldness of the legs, limb pain and weakness. The skin temperature examined in 28 girls with limb symptoms exhibited a slight decrease in the fingers (30.4±2.6 ) and a moderate decrease in the toes (27.1± 3.7 ). Digital plethysmograms revealed a reduced height of the waves, especially in the toes. The limb symptoms of four girls were compatible with the Japanese clinical diagnostic criteria for complex regional pain syndrome (CRPS), while those in the other 14 girls were consistent with foreign diagnostic criteria for CRPS. The Schellong test identified eight patients with orthostatic hypotension and four patients with postural orthostatic tachycardia syndrome. The girls with orthostatic intolerance and CRPS commonly experienced transient violent tremors and persistent asthenia. Electron-microscopic examinations of the intradermal nerves showed an abnormal pathology in the unmyelinated fibers in two of the three girls examined. Conclusion The symptoms observed in this study can be explained by abnormal peripheral sympathetic responses. The most common previous diagnosis in the studied girls was psychosomatic disease. The social problems of the study participants remained unresolved in that the severely disabled girls stopped going to school.

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Section: Orthostatic Intolerancementioning

confidence: 99%

Peripheral Sympathetic Nerve Dysfunction in Adolescent Japanese Girls Following Immunization with the Human Papillomavirus Vaccine

et al. 2014

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“…In patier~ts with orthostatic hypotension with blocked norepinephrine synthesis due to congenital deficiency of the enzyme dopamine beta hydroxylase, L-threodops increases blood pressure and reduces orthostatic hypotension (Biaggioni and Robertson, 1987;Man in 'T Veldt et al, 1987). In patients with other forms of autonomic failure, however, reports on the effect of L-threo-dops on blood pressure are conflicting (Suzuki etal., 1980(Suzuki etal., , 1981Birkmayer etal., 1983;Hoeldtke etal., 1984;Sakoda etal., 1985). Kachi etal.…”

Section: Introductionmentioning

confidence: 96%

Differential effect of L-threo-3,4-dihydroxyphenylserine in pure autonomic failure and multiple system atrophy with autonomic failure

Kaufmann

Oribe

Yahr

1991

J Neural Transm Gen Sect

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Treatment with L-threo-3,4-dihydroxyphenylserine (L-threo-dops), a synthetic precursor of norepinephrine, significantly increased upright blood pressure in patients with multiple system atrophy but had no effect on the upright blood pressure of patients with pure autonomic failure. These results suggest that the site of action of L-threo-dops is central and that its pressor effect requires intact peripheral sympathetic neurons.

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“…Plasma levels of norepinephrine were 0.082 ng/ml in the supine position, and 0.230 ng/ml for a 5-min stand, which are lower than normal (0.303 0.032 ng/ml, mean& SEM, in the supine position, and 0.519 ? 0.040 ng/ml for a 5-min stand) (1). The systolic pressure "overshoot" in Phase IV of the Valsalva maneuver was absent.…”

Section: Determination Of L-threo-dops and Norepinephrine In Plasmamentioning

confidence: 85%

“…Orthostatic hypotension is one of the most disabling manifestations in the disorder. To treat it, we adopted L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS), an immediate precursor amino acid of (-)-norepinephrine (1). L-threo-DOPS is enzymatically transformed to (-)-norepinephrine (Z), which exerts beneficial effects on the orthostatic symptoms based on norepinephrine depletion from the sympathetic nerve endings and denervation sypersensitivity of the receptors in the disorder (1).…”

mentioning

confidence: 99%

“…To treat it, we adopted L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS), an immediate precursor amino acid of (-)-norepinephrine (1). L-threo-DOPS is enzymatically transformed to (-)-norepinephrine (Z), which exerts beneficial effects on the orthostatic symptoms based on norepinephrine depletion from the sympathetic nerve endings and denervation sypersensitivity of the receptors in the disorder (1). The present paper describes neurocirculatory studies using a telemetry system for ambulatory intra-arterial recordings, in a patient with FAP whose orthostatic symptoms improved during 5 months of therapy with L-threo-DOPS.…”

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confidence: 99%

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Studies of neurocirculatory effects of long-term L-threo-3, 4-dihydroxyphenylserine administration in a patient with familial amyloidotic polyneuropathy

Azuma

Suzuki

Mizuno

et al. 1988

Acta Neurologica Scandinavica

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The case is reported of a 57-year-old woman with familial amyloidotic polyneuropathy and concomitant orthostatic hypotension for which L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS) was clinically effective. Testing of autonomic function under telemetric intra-arterial pressure monitoring before and during L-threo-DOPS treatment clearly demonstrated the pathophysiology of the sympathetic nervous system and its modification by L-threo-DOPS.

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Treatment of Orthostatic Hypotension in Familial Amyloid Polyneuropathy with L-Threo-3,4-Dihydroxyphenylserine

Cited by 3 publications

References 3 publications

Peripheral Sympathetic Nerve Dysfunction in Adolescent Japanese Girls Following Immunization with the Human Papillomavirus Vaccine

Peripheral Sympathetic Nerve Dysfunction in Adolescent Japanese Girls Following Immunization with the Human Papillomavirus Vaccine

Differential effect of L-threo-3,4-dihydroxyphenylserine in pure autonomic failure and multiple system atrophy with autonomic failure

Studies of neurocirculatory effects of long-term L-threo-3, 4-dihydroxyphenylserine administration in a patient with familial amyloidotic polyneuropathy

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