[Chronic progressive external ophthalmoplegia: clinical and electromyographic manifestations in a series of cases].
BACKGROUND
Chronic progressive external ophthalmoplegia (CPEO) is a common mitochondrial disease. The different conditions in this group of diseases overlap clinically, enzymatically and genetically. There is no effective treatment. Ptosis improves with corrective surgery involving tarsorrhaphy as a palliative measure.


METHODS
Code numbers were examined in a retrospective study conducted in order to search for patients with ptosis or ophthalmoplegia who had either visited or been admitted to the neurology department over the last 10 years. Data concerning these patients' clinical features and results of complementary tests were collected. Six patients with CPEO were identified, five of whom were females. Ages ranged from 44 to 72 years. All the patients had ptosis, although 50% were asymmetric. Half of them reported mild dysphagia while swallowing liquids. Levels of creatine phosphokinase and acetylcholine antireceptor antibodies were normal. Half the patients showed increased jitter and a muscle biopsy revealed that five of them had ragged red fibres. The most frequent enzyme deficit was complex I and IV deficiency. There were no familial forms; the most common genetic anomaly was single deletion in the mitochondrial deoxyribonucleic acid.


CONCLUSIONS
In cases of ptosis and ophthalmoplegia that do not respond to anticholinesterases, knowledge of this condition makes it possible to avoid the use of immunosuppressant drugs, which have important side effects.
Jiménez-Caballero
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PE
Servicio de Neurología, Hospital Virgen de la Salud, Toledo, España. pjimenez1010j@yahoo.es
Serviá
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M
Cabeza
C I
CI
Marsal-Alonso
C
C
Alvarez-Tejerina
A
A
D000328:Adult; D000368:Aged; D001706:Biopsy; D001763:Blepharoptosis / Q000209:etiology; D005117:Cardiac Complexes, Premature / Q000209:etiology; D030401:Cytochrome-c Oxidase Deficiency / Q000150:complications / Q000175:diagnosis; D003680:Deglutition Disorders / Q000209:etiology; D004576:Electromyography; D042967:Electron Transport Complex I / Q000032:analysis; D003576:Electron Transport Complex IV / Q000032:analysis; D005260:Female; D006327:Heart Block / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000201:enzymology / Q000473:pathology; D018656:Muscle Fibers, Fast-Twitch / Q000473:pathology; D009801:Oculomotor Muscles / Q000473:pathology; D017246:Ophthalmoplegia, Chronic Progressive External / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology*; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology
D003576:Electron Transport Complex IV; D042967:Electron Transport Complex I