Syndrome of continuous muscle fiber activity: case report with 11-year follow-up

Oliveira, José Teotônio de; Levy-Reis, Igor

doi:10.1590/S0004-282X1994000100019

Abstracts

A 16-year-old male patient who presented with muscle stiffness and dysphonia is described. Electromyography revealed continuous motor activity that was unaffected by peripheral nerve block or general anaesthesia, but was abolished by curare. The patient had a marked improvement after using phenytoin. The follow-up 11-years later corroborates with the proposed benignity of this syndrome, in spite of being dependent on medication.

muscle stiffness; continuous muscle fiber activity; Isaacs' syndrome

É relatado o caso de um paciente de 16 anos de idade do sexo masculino com quadro de rigidez muscular e disfonia. Eletromiografia revelou atividade motora contínua que não era alterada por bloqueio do nervo periférico ou anestesia geral, mas era abolida por curare. O paciente apresentou acentuada melhora após o uso de fenitoína. O seguimento do caso 11 anos mais tarde vem corroborar o proposto caráter benigno desta síndrome, apesar de o paciente ainda depender da medicação.

rigidez muscular; atividade de fibra muscular contínua; síndrome de Isaacs

Syndrome of continuous muscle fiber activity: case report with 11-year follow-up

Síndrome de atividade contínua da fibra muscular: relato de caso com seguimento de 11 anos

José Teotônio de Oliveira^I; Igor Levy-Reis^II

^INeurology Service, Hospital of Clinics, Federal University of Minas Gerais, Medical School, Belo Horizonte: Assistant Professor

^IINeurology Service, Hospital of Clinics, Federal University of Minas Gerais, Medical School, Belo Horizonte: Resident. Presented at the 15th Brazilian Congress of Neurology (Porto Alegre, October 1992)

SUMMARY

A 16-year-old male patient who presented with muscle stiffness and dysphonia is described. Electromyography revealed continuous motor activity that was unaffected by peripheral nerve block or general anaesthesia, but was abolished by curare. The patient had a marked improvement after using phenytoin. The follow-up 11-years later corroborates with the proposed benignity of this syndrome, in spite of being dependent on medication.

Key words: muscle stiffness, continuous muscle fiber activity, Isaacs' syndrome.

RESUMO

É relatado o caso de um paciente de 16 anos de idade do sexo masculino com quadro de rigidez muscular e disfonia. Eletromiografia revelou atividade motora contínua que não era alterada por bloqueio do nervo periférico ou anestesia geral, mas era abolida por curare. O paciente apresentou acentuada melhora após o uso de fenitoína. O seguimento do caso 11 anos mais tarde vem corroborar o proposto caráter benigno desta síndrome, apesar de o paciente ainda depender da medicação.

Palavras-chave: rigidez muscular, atividade de fibra muscular contínua, síndrome de Isaacs.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 22-setembro-1993.

Dr. José Teotônio de Oliveira - Serviço de Neurologia, Hospital das Clínicas UFMG - Av. Alfredo Balena 190 -30130-100 Belo Horizonte MG - Brasil.

1. Ashizawa T, Butler IJ, Harati Y, Rrongta SM. A dominantly inherited syndrome with continuous motor neuron discharges. Ann Neurol 1983, 13: 285-290.
2. Auger RG. Continuous muscle fiber activity. Sem Neurol 1991, 11: 258-266.
3. Barron SA, Heffner RR Jr. Continuous muscle fiber activity: a case with unusual clinical features. Arch Neurol 1979, 36: 520-521.
4. Coërs C, Telerman-Toppet N, Durdu J. Neurogenic benign fasciculations, pseudomyotonia and pseudotetany: a diasease in search for a name. Arch Neurol 1981, 38: 282-287.
5. Da Silva AB, Japp HH, Saldanha APD, Henriques FG. Síndrome de Isaacs: registro de um caso e revisão da literatura. Arq Neuropsiquiatr 1977, 35: 139-145.
6. Fraser JL, Layzer RB, Olney RK. Muscle stiffness due to complex repetitive discharges (Abtr). Neurology 1992, 42 (Suppl 3): 337.
7. Garcia-Merino A, Cabello A, Mora J, Liaño H. Continuous muscle fiber activity, peripheral neuropathy and thymoma. Ann Neurol 1991, 29: 215-218.
8. Gardner-Medwin D, Walton JN. Myokymia with impaired muscular relaxation. Lancet 1969, 7586: 127-130.
9. Hahn AF, Parkes AW, Bolton CF, Stewart SA. Neuromyotonia in hereditary motor neuropathy. J Neurol Neurosurg Psychiatry 1991, 54: 230-235.
10. Hanson PA, Martinez LB, Cassidy R. Contractures, continuous muscle discharges and titubation. Ann Neurol 1977, 1: 120-124.
11. Harik SI, Baraka AS, Tomeh GF, Mire-Salman J, Kronful Z, Afifi AK. Autonomous peripheral nerve activity causing generalized muscle stiffness and fasciculations: report of a case with physiological, pharmacological, and morphological observations. Johns Hopkins Med 1976, 139: 49-60.
12. Hughes RC, Matthews WB. Pseudo-myotonia and myokymia. J Neurol Neurosurg Psychiatry, 1969, 32: 11-14.
13. Isaacs H. A syndrome of continuous muscle-fibre activity. J Neurol Neurosurg Psychiatry 1961,24:319-325.
14. Isaacs H, Heffron JJ. The syndrome of continuous muscle-fiber acticity cured: further studies. J Neurol Neurosurg Psychiatry 1974, 37: 1231-1235.
15. Lance JW, Burke D, Pollard J. Hyperexcitability of motor and sensory neurons in neuromyotonia. Ann Neurol 1979, 5: 523-532.
16. Levy JA, Wittig EO, Ferraz ECF. Esclerodermia associada a atividade elétrica muscular contínua. Arq Neuropsiquiatr 1965, 23: 283-288.
17. McGuirre SA, Tomasovic JJ, Adkerman N Jr. Hereditary continuous muscule fiber activity. Arch Neurol 1984, 41: 395-396.
18. Mitsremotor H, Wilbourn AJ, Subramony SH. Generalized myokymia and gold therapy. Arch Neurol 1982,39: 449-450.
19. Oda K, Fukushima N, Shibasaki H, Ohnishis A. Hypoxia sensitive hyperexcitability of the intramuscular nerve axons in Isaac's syndrome. Ann Neurol 1989, 25:140-145.
20. Reeback J, Benton S, Swash M, Schwartz MS. Penicillamine induced neuromyotonia. Br Med J 1979, 6176: 1464-1465.
21. Sakai T, Hosokawa S, Shibasaki H, Goto I, Kuroiwa Y, Sonoda H, Murai Y. Syndrome of continuous muscle-fiber activity: increased CSF GABA and effect of dantrolene. Neurology 1983, 33:495-498.
22. Sinha S, Newsom-Davis J, Milk K, Byrne N, Lange B, Vincent A. Autoimmune aetiology for acquired neuromyotonia (Isaac's syndrome). Lancet 1991, 338: 75-77.
23. Tahmoush AJ, Alonso RJ, Tahmoush GP, Heiman-Patterson TD. Cramp-fasciculation syndrome: a treatable hyperexcitable peripheral nerve disorder. Neurology 1991, 41: 1021-1024.
24. Teive HG, Arruda WO, Bittar NE, Gorz AM, Minguetti G. Síndrome de Issacs: relato de um caso. Arq Neuropsiquiatr 1988, 46: 182-186.
25. Thompson PD. Stiffmuscles. J Neurol Neurosurg Psychiatry 1993, 56: 121-124.
26. Wallis WE, Von Poznack A, Plum E Generalized muscular stiffness, fasciculations, and myokymia of peripheral nerve origin. Arch Neurol 1970, 22: 430-439.
27. Walsh JC. Neuromyotonia: an unusual presentation of intrathoracic malignancy. J Neurol Neurosurg Psychiatry 1976, 39: 1086-1091.
28. WarmoIts JR, Mendell JR. Neurotonia: impulse-induced repetitive discharges in a motor nerves in peripheral neuropathy. Ann Neurol 1980, 7: 245-250.
29. Welch LK, Appenzeller O, Bicknell JM. Peripheral neuropathy with myokymia sustained muscular contraction, and continuous motor unit activity. Neurology 1972, 22: 161-169.
30. Wilton A. 21-year follow-up of myokymia with impaired muscle relaxation. Lancet 1990, 336: 1138-1139.
31. Zisfein J, Sivak M, Aron AM, Bender AN. Issac's syndrome with muscle hypertrophy reversed by phenytoin therapy. Arch Neurol 1983, 40: 241-242.

Publication Dates

Publication in this collection
19 Jan 2011
Date of issue
Mar 1994

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Ashizawa T, Butler IJ, Harati Y, Rrongta SM. A dominantly inherited syndrome with continuous motor neuron discharges. Ann Neurol 1983, 13: 285-290.

[2] 2. Auger RG. Continuous muscle fiber activity. Sem Neurol 1991, 11: 258-266.

[3] 3. Barron SA, Heffner RR Jr. Continuous muscle fiber activity: a case with unusual clinical features. Arch Neurol 1979, 36: 520-521.

[4] 4. Coërs C, Telerman-Toppet N, Durdu J. Neurogenic benign fasciculations, pseudomyotonia and pseudotetany: a diasease in search for a name. Arch Neurol 1981, 38: 282-287.

[5] 5. Da Silva AB, Japp HH, Saldanha APD, Henriques FG. Síndrome de Isaacs: registro de um caso e revisão da literatura. Arq Neuropsiquiatr 1977, 35: 139-145.

[6] 6. Fraser JL, Layzer RB, Olney RK. Muscle stiffness due to complex repetitive discharges (Abtr). Neurology 1992, 42 (Suppl 3): 337.

[7] 7. Garcia-Merino A, Cabello A, Mora J, Liaño H. Continuous muscle fiber activity, peripheral neuropathy and thymoma. Ann Neurol 1991, 29: 215-218.

[8] 8. Gardner-Medwin D, Walton JN. Myokymia with impaired muscular relaxation. Lancet 1969, 7586: 127-130.

[9] 9. Hahn AF, Parkes AW, Bolton CF, Stewart SA. Neuromyotonia in hereditary motor neuropathy. J Neurol Neurosurg Psychiatry 1991, 54: 230-235.

[10] 10. Hanson PA, Martinez LB, Cassidy R. Contractures, continuous muscle discharges and titubation. Ann Neurol 1977, 1: 120-124.

[11] 11. Harik SI, Baraka AS, Tomeh GF, Mire-Salman J, Kronful Z, Afifi AK. Autonomous peripheral nerve activity causing generalized muscle stiffness and fasciculations: report of a case with physiological, pharmacological, and morphological observations. Johns Hopkins Med 1976, 139: 49-60.

[12] 12. Hughes RC, Matthews WB. Pseudo-myotonia and myokymia. J Neurol Neurosurg Psychiatry, 1969, 32: 11-14.

[13] 13. Isaacs H. A syndrome of continuous muscle-fibre activity. J Neurol Neurosurg Psychiatry 1961,24:319-325.

[14] 14. Isaacs H, Heffron JJ. The syndrome of continuous muscle-fiber acticity cured: further studies. J Neurol Neurosurg Psychiatry 1974, 37: 1231-1235.

[15] 15. Lance JW, Burke D, Pollard J. Hyperexcitability of motor and sensory neurons in neuromyotonia. Ann Neurol 1979, 5: 523-532.

[16] 16. Levy JA, Wittig EO, Ferraz ECF. Esclerodermia associada a atividade elétrica muscular contínua. Arq Neuropsiquiatr 1965, 23: 283-288.

[17] 17. McGuirre SA, Tomasovic JJ, Adkerman N Jr. Hereditary continuous muscule fiber activity. Arch Neurol 1984, 41: 395-396.

[18] 18. Mitsremotor H, Wilbourn AJ, Subramony SH. Generalized myokymia and gold therapy. Arch Neurol 1982,39: 449-450.

[19] 19. Oda K, Fukushima N, Shibasaki H, Ohnishis A. Hypoxia sensitive hyperexcitability of the intramuscular nerve axons in Isaac's syndrome. Ann Neurol 1989, 25:140-145.

[20] 20. Reeback J, Benton S, Swash M, Schwartz MS. Penicillamine induced neuromyotonia. Br Med J 1979, 6176: 1464-1465.

[21] 21. Sakai T, Hosokawa S, Shibasaki H, Goto I, Kuroiwa Y, Sonoda H, Murai Y. Syndrome of continuous muscle-fiber activity: increased CSF GABA and effect of dantrolene. Neurology 1983, 33:495-498.

[22] 22. Sinha S, Newsom-Davis J, Milk K, Byrne N, Lange B, Vincent A. Autoimmune aetiology for acquired neuromyotonia (Isaac's syndrome). Lancet 1991, 338: 75-77.

[23] 23. Tahmoush AJ, Alonso RJ, Tahmoush GP, Heiman-Patterson TD. Cramp-fasciculation syndrome: a treatable hyperexcitable peripheral nerve disorder. Neurology 1991, 41: 1021-1024.

[24] 24. Teive HG, Arruda WO, Bittar NE, Gorz AM, Minguetti G. Síndrome de Issacs: relato de um caso. Arq Neuropsiquiatr 1988, 46: 182-186.

[25] 25. Thompson PD. Stiffmuscles. J Neurol Neurosurg Psychiatry 1993, 56: 121-124.

[26] 26. Wallis WE, Von Poznack A, Plum E Generalized muscular stiffness, fasciculations, and myokymia of peripheral nerve origin. Arch Neurol 1970, 22: 430-439.

[27] 27. Walsh JC. Neuromyotonia: an unusual presentation of intrathoracic malignancy. J Neurol Neurosurg Psychiatry 1976, 39: 1086-1091.

[28] 28. WarmoIts JR, Mendell JR. Neurotonia: impulse-induced repetitive discharges in a motor nerves in peripheral neuropathy. Ann Neurol 1980, 7: 245-250.

[29] 29. Welch LK, Appenzeller O, Bicknell JM. Peripheral neuropathy with myokymia sustained muscular contraction, and continuous motor unit activity. Neurology 1972, 22: 161-169.

[30] 30. Wilton A. 21-year follow-up of myokymia with impaired muscle relaxation. Lancet 1990, 336: 1138-1139.

[31] 31. Zisfein J, Sivak M, Aron AM, Bender AN. Issac's syndrome with muscle hypertrophy reversed by phenytoin therapy. Arch Neurol 1983, 40: 241-242.