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Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2019

Open Access 01-12-2019 | Alopecia | Review

Treatment of Satoyoshi syndrome: a systematic review

Published in: Orphanet Journal of Rare Diseases | Issue 1/2019

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Abstract

Background

Satoyoshi syndrome is a multisystemic rare disease of unknown etiology, although an autoimmune basis is presumed. Its main symptoms are: painful muscle spasms, diarrhea, alopecia and skeletal abnormalities. Clinical course without treatment may result in serious disability or death. A review of treatment and its response is still pending.

Results

Sixty-four cases of Satoyoshi syndrome were published between 1967 and 2018. 47 cases described the treatment administered. Drugs used can be divided into two main groups of treatment: muscle relaxants/anticonvulsants, and corticosteroids/immunosuppressants. Dantrolene improved muscle symptoms in 13 out of 15 cases, but not any other symptoms of the disease. Other muscle relaxants or anticonvulsant drugs showed little or no effect. 28 out of 30 cases responded to a regimen that included costicosteroids. Other immunosuppressive drugs including cyclosporine, mycophenolate mofetil, azathioprine, methotrexate, tacrolimus and cyclophosphamide were used to decrease corticosteroid dose or improve efficacy. Immunoglobulin therapy was used in nine patients and four of them obtained a favorable response.

Conclusion

Corticosteroids was the most widely treatment employed with the best results in Satoyoshi syndrome. Further studies are needed to determine optimal dose and duration of corticosteroids as well as the role of other immunosuppressants and immunoglobulin therapy. Genetic or autoimmune markers will be useful to guide future therapies.
Literature
1.
Satoyoshi E. A síndrome of progressive muscle spasm, alopecia and diarrhea. Neurology. 1978;28:458–71.PubMedCrossRef
2.
Castiglioni C, Díaz A, Moënne K, Mericq V, Salvador F, Hernández C. Síndrome de Satoyoshi: Enfermedad multisistémica con respuesta exitosa a tratamiento esteroidal. Rev Méd Chile. 2009;137:542–6.PubMedCrossRef
3.
Arita J, Hamano S, Nara T, Maekawa K. Intravenous gammaglobulin therapy of Satoyoshi syndrome. Brain Dev. 1996;18:409–11.PubMedCrossRef
4.
Satoyoshi E, Yamada K. Recurrent muscle spasm of central origin. A report of two cases. Arch Neurol. 1967;16:254–64.PubMedCrossRef
5.
Rudnicka L, Kwiatkowska M,3 Rakowska A, Czuwara J,Olszewska M. Alopecia areata. How not to miss Satoyoshi syndrome? J Dermatol 2014; 41: 951–956.
6.
Ikeda K, Satoyoshi E, Kinoshita M, Wakata N, Iwasaki Y. Satoyoshi’s Syndrome in an adult: a review of the literature of adult onset cases. Intern Med. 1998;37:784–7.PubMedCrossRef
7.
Ikegawa S, Nagano A, Satoyoshi E. Skeletal abnormalities in Satoyoshi's syndrome: a radiographic study of eight cases. Skelet Radiol. 1993;22:321–4.CrossRef
8.
Ishihara M, Ogawa K, Suzuki Y, Kamei S, Ochiai t SM. Adult-onset Satoyoshi Syndrome with prominent laterality of clinical features. Intern Med. 2014;53:2811–6.PubMedCrossRef
9.
Uddin ABMS, Walters AS, Ali A, Brannan T. A unilateral presentation of “Satoyoshi syndrome”. Parkinsonism Relat Disord. 2002;8:211–3.PubMedCrossRef
10.
11.
12.
13.
Merino de Paz N, Guimera Martin-Neda F, Rodriguez Martin M, Saez Rodriguez M, Merino de Paz M, Contreras Ferrer P, Pitti Perez R. Diphencyprone in the treatment of Satoyoshi síndrome. 72nd annual meeting of the American Academy of Dermatology. J Am Acad Dermatol 2014; 70 (5-Su 1): AB 89.
14.
Rudnicka L, Kwiatkowska M, Rakowska A, Olszewska M. Diagnostic criteria for Satoyoshi síndrome. J Am Acad Dermatol. 2015;72(5 - SU 1):AB112.
15.
Meneni SA. Satoyoshi syndrome in pregnancy. BJOG-An International Journal of Obstetrics and Gynaecology. 2016;123(SU 1):30.
16.
Drost G, Verrips A, van Steensel MAM, Baarsma R, Zwarts MJ. Painful muscle contraction in Satoyoshi syndrome: cramp or spasm? Neuromuscul disord 7th International Congress of the World-Muscle-Society. 2002;12(7–8):772.
17.
Sunohara N. Satoyoshi syndrome (disease)Ryoikibetsu Shokogun Shirizu. 2001;36:384–8.
18.
Saitoh S. Satoyoshi syndrome. Ryoikibetsu Shokogun Shirizu. 2001;34 (part 2 "Pt 2"):600–1.
19.
Dong M, Wei M, Zhao SM, Shi HP, Nu DJ, Yu W. Satoyoshi's syndrome in a case. Zhonghua er ke za zhi Chinese journal of pediatrics. 2004;42:224.PubMed
20.
Arimura K. Isaacs' syndrome, stiff person syndrome and Satoyoshi disease: Pathomechanisms and treatment. Clinical Neurology. 2004;44:805–7.PubMed
21.
Satoyoshi E. [Satoyoshi\'s syndrome: a syndrome of progressive muscle spasm, alopecia, and diarrhea]. Nippon rinsho. Japanese. J Clin Med. 1990;48:1540–6.
22.
Satoyoshi E. Satoyoshi's disease (a syndrome of progressive muscle spasms) Nippon rinsho. Japanese journal of clinical medicine. 1978;Suppl:1744–5.
23.
Satoyoshi E. Muscle cramps (Satoyoshi's disease). Nippon rinsho. Japanese journal of clinical medicine. 1977;35(Suppl 1):664–5.PubMed
24.
del Castillo V, Altamirano N, Rasmussen A, Esmer C, Ruiz M. Bone mineral metabolism in Satoyoshi syndrome associated with autoimmune thyroiditis. Am J Human Gen. 2000;67(4):SU2–109.
25.
Satoyoshi E. Generalized Komuragaeri disease. A syndrome of progressive myospasm gravis, alopecia and diarrhea (Japanese). Advances in Neurological Sciences. 1976;20(4):681–9.
26.
Keyser A, Horstink MWIM, Joosten EMG. A new case of Satoyoshi's syndrome. In: World congress of neurology. International congress series 548. Amsterdam: Excepta Medica; 1981. p. 396.
27.
Tajima Y, Tashiro K, Gotoh Y. Satoyoshi disease (generalized komuragaeri disease) associated with bilateral gastrocnemius hypertrophy. Shinkei Naika. Neurol Med. 1994;41:93–4.
28.
Komatsu M, Kimura S, Tanaka M, Morimatsu M, Hirai S. Generalized recurrent muscle spasm (Satoyoshi) and the effect of hypernatremia on the spasm: a case report. Shinkei Naika (Neurol Med). 1984;21:521–3.
29.
Nanba R, Hayabara T, Makihata H, Ikeda H. A case of "syndrome of recurrent muscle spasm (Satoyoshi)" with reference to the effect of dantrolene sodium on the spasm. Shinkei Naika (Neurol Med). 1997;7:154.
30.
Okamoto K, Tanaka M, Kouki M, Hirai S. A case of generalized komuragaeri disease (Satoyoshi). Shinkei Naika, vol. 14; 1981. p. 50–6.
31.
Higa H, Kawane K, Kawahira M, et al. A case of recurrent generalized muscle spasm with reduced IgG content in the serum. Clin Neurol. 1978;18:157.
32.
Merello M, Garcia H, Nogués M, Leiguarda R. Masticatory muscle spasm in a non-Japanese patient with Satoyoshi Syndrome successfully treated with botulinum toxin. Mov Disord. 1994;9:104–5.PubMedCrossRef
33.
McKinley K, Harati Y, Fishman M, Parke J, Barroso A. Satoyoshi syndrome without malabsorption or response to immunosuppression. Ann Neurol. 1991;30(2):264.
34.
35.
Ashalatha R, Kishore A, Sarada C, Nair MD. Satoyoshi syndrome. Neurol India. 2004;52:94–5.PubMed
36.
Aver’ianov IN, Vodolagin VD, Logunova LV, LIa L. Positive therapeutic effect of diacarb in the syndrome of progressive muscle spasms, alopecia and diarrhea (Satoyoshi syndrome). Zh Nevropatol Psikhiatr Im S S Korsakova. 1984;84:1623–7.PubMed
37.
38.
Merino de Paz N, Rodriguez-Martin M, Contreras Ferrer P, Pestana Eliche M, Noda Cabrera A. Satoyoshi Syndrome: a cause of alopecia Universalis in association with neurologic and bony abnormalities. Pediatr Dermatol. 2013;30:e22–4.PubMedCrossRef
39.
Venegas-Vega CA, Rivera-Vega MR, Cuevas-Covarrubias S, Orozco J, Kofman-Alfaro S. Satoyoshi syndrome with unusual skeletal abnormalities and parental consanguinity. Am J Med Genet Part A. 2009;149A:2448–51.PubMedCrossRef
40.
De-Xin W, Hui-di F. Three cases of recurrent generalized muscle spasm in China. Jap J Med. 1985;24:263–8.CrossRef
41.
Li J, Peng D, Jiang T, Avivi-Arber L. Satoyoshi syndrome with progressive orofacial manifestations: a case history report. Int J Prosthodont. 2017;30:163–7.PubMedCrossRef
42.
Kuru S, Riku S, Nakayabu M, Kobayashi Y, Ieda T. A case of 'syndrome of progressive muslce spasm, alopecia, and diarrhea (Satoyoshi)' treated with steroid pulse therapy. Clinical Neurology. 1992;32(6):612–5.PubMed
43.
Matsumura T, Yokoe M, Shinno S. [A case of Satoyoshi syndrome complicating marginal gingivitis of the mandible and dislocation of the temporomandibular joint]. Japanese. PubMed PMID: 12710092.
44.
Ikegawa S, Nagano A, Nakamura K, Kurokawa T. A case of Satoyoshi’s Syndrome. J Pediatr Orthop. 1993;13:793–6.PubMedCrossRef
45.
Matsuo N, Fujioka M, Tsuchiya Y, Cho H, Nagai T, Kumagai M. Multiple metaphyseal lesions in a child with a syndrome of progressive muscle cramps, alopecia and stunted growth (Satoyoshi disease). Radiation Medicine - Medical Imaging and Radiation Oncology. 1983;1:205–7.
46.
Ishii K, Furusho K, Tamaoka A, Morishita Y. Lymphocytic colitis in Satoyoshi Syndrome. South Med J. 2010;103:591.PubMedCrossRef
47.
Rosales, RL; Banzon, JE. Painful muscle spasms, twisting, and loose stools: what a combination in an adolescent girl!. Movement disorders: unforgettable cases and lessons from the bedside. ISBN: 978-1-9362-8728-4. 2013. 217–221.
48.
Satoh A, Yoshimura T, Mori M, Tsujihata M, Takamori M. A case of generalized Komuragaeri disease complicating myasthenia gravis. Clin Neurol. 1982;22:251–7.
49.
Inoue K. General muscle crapms (Komuragaeri disease) and its intestinal absortion. Clin Neurol. 1976;20:699–705.
50.
Endo K, Yamamoto T, Nakamura K, Hoshi A, Yamanoi T, Watanabe A, Homma M. Improvement of Satoyoshi syndrome with tacrolimus and corticosteroids. Neurology. 2003;60(12):2014–5 PubMed PMID: 12821760.PubMedCrossRef
51.
Asherson RA, Giampaolo D, Strimling M. A case of adult-onset Satoyoshi syndrome with gastric ulceration and eosinophilic enteritis. Nat Clin Pract Rheumatol. 2008;4:439–44.PubMedCrossRef
52.
Adachi H, Riku S, Fujishiro K, Kuru S. A case of Satoyoshi syndrome with symptoms resembling neuroleptic malignant syndrome. Rinsho Shinkeigaku. 1998;38(7):637–40.PubMed
53.
Mani V, George R. Satoyoshi syndrome - a case report from India. Pediatr Dermatol. 2017;00:1–3.
54.
Sharpe A, Mahadasu S, Manda P, Meneni D. Satoyoshi syndrome in pregnancy. Eur J Obstet Gynecol Reprod Biol. 2016;199:215–6.PubMedCrossRef
55.
Drost G, Verrips A, van Engelen BG, Stegeman DF, Zwarts MJ. Involuntary painful muscle contractions in Satoyoshi syndrome: a surface electromyographic study. Mov Disord. 2006;21(11):2015–8.PubMedCrossRef
56.
Ehlayel MS, Lacassie Y. Satoyoshi Syndrome: an unusual postnatal multisystemic disorder. Am J Med Genet. 1995;57:620–5.PubMedCrossRef
57.
Yamagata T, Miyao M, Momoi M, Matsumoto S, Yanagisawa M. A case of generalized komuragaeri disease (Satoyoshi disease) treated with glucocorticoid. Clin Neurol. 1991;31:79–83.
58.
Ezgu FS, Tumer L, Serdaroglu A, Hasanoglu A, Cansu A, Hirfanoglu T, Dalgic B. The co-existence of Satoyoshi syndrome and myoadenylate deaminase deficiency. J Inherit Metab Dis. 2005;28:253.
59.
Montanaro VVA, Hora TF, Couto CM, Ribas FD. Adult-onset Satoyoshi syndrome in a young male. Neuromuscul Disord. 2017;27:382–38.PubMedCrossRef
60.
Dhamija R, Renaud DL, Pittock SJ, McKeon A, Lachance DH, Nickels KC, Wirrell EC, Kuntz NL, King MD, Lennon VA. Neuronal voltage-gated Potassium Channel complex autoimmunity in children. Pediatr Neurol. 2011;44:275–81.PubMedCrossRef
61.
Cecchin CR, Félix TM, Magalhães RB, Furlanetto TW. Satoyoshi Syndrome in a Caucasian girl improved with glucocorticoids. Am J Med Genet A. 2003;118A:52–4.PubMedCrossRef
62.
Solera J, Álvarez S, Botet J, de Cabo C. A newly homozygous variant in ZNF808: a possible candidate gene for Satoyoshi Syndrome? J Neurol Sci. 2017;379:226–8.PubMedCrossRef
63.
Heger S, Kuester RM, Volk R, Stephani U, Sippell WG. Satoyoshi syndrome: a rare multisystemic disorder requiring systemic and symptomatic treatment. Brain Dev. 2006;28:300–4.PubMedCrossRef
64.
Kamat D, Petry L, Berry S. A case of Satoyoshi Syndrome: a multisystem disorder. Clin Pediatr (Phila). 2003;42:745–8.CrossRef
65.
Wisuthsarewong W, Likitmaskul S, Manonukul J. Satoyoshi syndrome. Pediatr Dermatol. 2001;18:406–10.PubMedCrossRef
66.
67.
Son KR, JH KK, Kim BJ, Kim SJ, Ma JS. A case of Satoyoshi syndrome presented with progressive muscular spasm and alopecia. J Korean Pediatr Soc. 2002;45:1165–9.
68.
NagahamaT YK, Oishi T, Iwashita A, Hirai F, Yao T, Matsui T, Takaki Y. GI manifestations of Satoyoshi’s syndrome. Gastrointest Endosc. 2006;64:143–5.CrossRef
69.
Solera J, Rallo B, Herranz AS, Pardal JM, Martin-del Rio R, de Cabo C. High glycine levels in the cerebrospinal fluid in Satoyoshi syndrome. J Neurol Sci. 2015;357(1–2):312–3.PubMedCrossRef
70.
Pardal-Fernández JM, Solera-Santos J, Iniesta-López I, Rodríguez-Vázquez M. Satoyoshi's syndrome related muscle spasms: functional study. Rev Neurol (Paris). 2012 Mar;168(3):291–5.CrossRef
71.
Li J, Jiang T, Feng HL. A case report of dental abnormality and prosthetic treatment of Satoyoshi syndrome. Zhonghua Kou Qiang Yi Xue Za Zhi. 2008;43(4):213–5.PubMed
72.
Drost G, Verrips A, Hooijkaas H, Zwarts M. Glutamic acid decarboxylase antibodies in Satoyoshi syndrome. Ann Neurol. 2004;55(3):450–1.PubMedCrossRef
73.
Haymon M, Willis RB, Ehlayel MS, Lacassie Y. Radiological and orthopedic abnormalities in Satoyoshi syndrome. Pediatr Radiol. 1997 May;27(5):415–8.PubMedCrossRef
74.
Satoh A, Tsujihata M, Yoshimura T, Mori M, Nagataki S. Myasthenia gravis associated with Satoyoshi syndrome: muscle cramps, alopecia, and diarrhea. Neurology. 1983;33(9):1209–11.PubMedCrossRef
75.
Matsuura E, Matsuyama W, Sameshima T, Arimura K. Satoyoshi syndrome has antibody against brain and gastrointestinal tissue. Muscle Nerve. 2007;36(3):400–3.PubMedCrossRef
76.
Itahara K, Oyama K, Ohara Y, Kobayashi K, Tsumuraya K, Iijima K, Nakamura S, Rikimaru S. Gastroscopic findings of a patient with recurrent muscle spasm of central origin (Satoyoshi). Clin Neurol. 1976;20:126–8.
77.
78.
79.
Endo K, Kumagai T, Nakahara T, Nakamura K, Shimizu M, Watanabe A, et al. A novel autoantibody associated with Satoyoshi syndrome. Neuroimmunology (Tokyo). 2001;9:102–3.
Metadata
Title
Treatment of Satoyoshi syndrome: a systematic review
Publication date
01-12-2019

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