Malignant ventricular arrhythmia in a case of adult onset of spinal muscular atrophy (kugelberg-welander disease)

Journal of Cardiovascular Electrophysiology

Volumn 20, Issue 3, 2009, Pages 342-344

  Roos, Markus ^a   Sarkozy, Andrea ^a   Chierchia, Gian Battista ^a   De Wilde, Philippe ^a   Schmedding, Eric ^a   Brugada, Pedro ^a  

^a UNIVERSITY HOSPITAL BRUSSELS   (Belgium)

Author keywords

Atrioventricular block; Cardioverter defibrillator; Kugelberg Welander disease; Spinal muscular atrophy; Ventricular tachycardia

Indexed keywords

BETA ADRENERGIC RECEPTOR BLOCKING AGENT;

ADULT; ARTICLE; CASE REPORT; CLINICAL FEATURE; DEFIBRILLATOR; DISEASE COURSE; ELECTROCARDIOGRAPHY MONITORING; ELECTROMYOGRAPHY; FAMILY HISTORY; FOLLOW UP; HEART ATRIUM PACEMAKER; HEART ELECTROPHYSIOLOGY; HEART VENTRICLE ARRHYTHMIA; HIS BUNDLE; HOLTER MONITORING; HUMAN; KUGELBERG WELANDER DISEASE; MALE; ONSET AGE; PATIENT ASSESSMENT; PRIORITY JOURNAL; SPINAL MUSCULAR ATROPHY;

ADULT; CARDIAC PACING, ARTIFICIAL; HUMANS; MALE; SPINAL MUSCULAR ATROPHIES OF CHILDHOOD; TACHYCARDIA, VENTRICULAR; TREATMENT OUTCOME;

EID: 60849117556     PISSN: 10453873     EISSN: 15408167     Source Type: Journal    
DOI: 10.1111/j.1540-8167.2008.01327.x     Document Type: Article

References (14)

1. Lefebvre S, Burglen L, Reboullet S, Clermont O, Burlet P, Viollet L, Benichou B, Cruaud C, Millasseau P, Zeviani M : Identification and characterization of a spinal muscular atrophy-determining gene. Cell 1995 80 : 155 165.
2. Roy N, Mahadevan MS, McLean M, Shutler G, Yaraghi Z, Farahani R, Baird S, Besner-Johnston A, Lefebvre C, Kang X : The gene for neuronal apoptosis inhibitory protein is partially deleted in individuals with spinal muscular atrophy. Cell 1995 80 : 167 178.
3. Munsat TL, Davies KE : International SMA consortium meeting (26-28 June 1992, Bonn, Germany). Neuromuscul Disord 1992 2 : 423 428.
4. Distefano G, Sciacca P, Parisi MG, Parano E, Smilari P, Marletta M, Fiumara A : Heart involvement in progressive spinal muscular atrophy. A review of the literature and case histories in childhood. Pediatr Med Chir 1994 16 : 125 128.
5. Zerres K, Davies KE : 59th ENMC International Workshop: Spinal muscular atrophies: Recent progress and revised diagnostic criteria (17-19 April 1998, Soestduinen, The Netherlands). Neuromuscul Disord 1999 9 : 272 278.
6. Wirth B : An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA). Hum Mutat 2000 15 : 228 237.
7. Zerres K, Rudnik-Schoneborn S: 93rd ENMC international workshop: Non-5q-spinal muscular atrophies (SMA) - clinical picture (6-8 April 2001, Naarden, The Netherlands). Neuromuscul Disord 2003 13 : 179 183.
8. Garvie JM, Woolf AL : Kugelberg-Welander syndrome (hereditary proximal spinal muscular atrophy). BMJ 1966 1 : 1458 1461.
9. Kugelberg E, Welander L : Heredofamilial juvenile muscular atrophy simulating muscular dystrophy. AMA Arch Neurol Psychiatry 1956 75 : 500 509.
10. Elkohen M, Vaksmann G, Elkohen MR, Francart C, Foucher C, Rey C : Cardiac involvement in Kugelberg-Welander disease. A prospective study of 8 cases. Arch Mal Coeur Vaiss 1996 89 : 611 617.
11. Takahashi N, Shimada T, Ishibashi Y, Sugamori T, Hirano Y, Oyake N, Murakami Y : Cardiac involvement in Kugelberg-Welander disease: A case report and review. Am J Med Sci 2006 332 : 354 356.
12. Tanaka H, Uemura N, Toyama Y, Kudo A, Ohkatsu Y : Cardiac involvement in the Kugelberg-Welander syndrome. Am J Cardiol 1976 38 : 528 532.
13. Tanaka H, Nishi S, Nuruki K, Tanaka N : Myocardial ultrastructural changes in Kugelberg-Welander syndrome. Br Heart J 1977 39 : 1390 1393.
14. Sterz H, Harrer G, Marchet H, Kaserer HP, Schlamberger H, Samec H, Stark U : Primary and neurogenic skeletal muscle diseases or paralysis with marked disturbances of cardiac rhythm. Z Kreislaufforsch 1971 60 : 1 13.