ABSTRACT
INTRODUCTION: A peculiar feature of seronegative myasthenia gravis is that it presents negative acetylcholine-receptor antibodies; determination of muscle-specific receptor tyrosine kinase (MuSK) antibodies defines a subgroup of patients with generalised myasthenia gravis with certain clinical and neurophysiological peculiarities. DEVELOPMENT: Its diagnosis requires the presence of weakness with fatigability, determination of positive anti-MuSK antibodies and alterations in neurophysiological testing of the neuromuscular junction. It is usually more serious and has a poorer prognosis than the seropositive forms, develops in an acute or subacute manner, and the neurological deficit predominates in the facial, bulbar and respiratory muscles. CONCLUSIONS: Titration of the anti-MuSK antibodies and conducting neurophysiological tests, especially jitter assessment using single-fibre electromyography in clinically deficient muscles, are not only necessary for an early diagnosis of these clinical forms, but also so as to be able to carry out an objective evaluation of the clinical progression and response to treatment.
Subject(s)
Myasthenia Gravis/diagnosis , Antibodies/blood , Humans , Myasthenia Gravis/blood , Receptor Protein-Tyrosine Kinases/immunology , Receptors, Cholinergic/immunologyABSTRACT
La miastenia grave generalizada seronegativa tiene la peculiaridad de presentar anticuerpos contrael receptor de la acetilcolina negativos; sin embargo, la determinación de anticuerpos contra el receptor de tirosincinasa muscular específica (MuSK) define un subgrupo de pacientes con miastenia grave generalizada con peculiaridades desde un punto de vista clínico y neurofisiológico. Desarrollo. Para su diagnóstico, es necesaria la presencia de debilidad con fatiga,determinación de anticuerpos anti-MuSK positivos y pruebas neurofisiológicas de placa neuromuscular alteradas. Suele ser clínicamente más grave y con peor pronóstico que las formas seropositivas, cursa de forma aguda o subaguda y el déficit neurológicopredomina en la musculatura facial, bulbar y respiratoria. Conclusión. La titulación de los anticuerpos anti-MuSK y la realización de pruebas neurofisiológicas, especialmente la valoración del jitter con electromiografía de fibra simple enmúsculos clínicamente deficitarios, no sólo son necesarias para el diagnóstico precoz de estas formas clínicas, sino también para valorar de forma objetiva la evolución clínica y la respuesta al tratamiento
A peculiar feature of seronegative myasthenia gravis is that it presents negative acetylcholine-receptorantibodies; determination of muscle-specific receptor tyrosine kinase (MuSK) antibodies defines a subgroup of patients with generalised myasthenia gravis with certain clinical and neurophysiological peculiarities. Development. Its diagnosis requires the presence of weakness with fatigability, determination of positive anti-MuSK antibodies and alterations in neurophysiological testing of the neuromuscular junction. It is usually more serious and has a poorer prognosis than the seropositive forms, develops in an acute or subacute manner, and the neurological deficit predominates in the facial, bulbar and respiratory muscles. Conclusions. Titration of the anti-MuSK antibodies and conducting neurophysiological tests, especially jitter assessment using single-fibre electromyography in clinically deficient muscles, are not only necessary for an early diagnosis of these clinical forms, but also so as to be able to carry out an objective evaluation of the clinical progression and response to treatment
Subject(s)
Humans , Myasthenia Gravis/diagnosis , Electromyography , Receptor Protein-Tyrosine Kinases/analysis , Receptors, Cholinergic/deficiencySubject(s)
Analgesics, Opioid/adverse effects , Benzodiazepines/adverse effects , Globus Pallidus/pathology , Heroin/adverse effects , Hypoxia, Brain , Respiratory Insufficiency/chemically induced , Adult , Female , Humans , Hypoxia, Brain/etiology , Hypoxia, Brain/pathology , Magnetic Resonance Imaging , Tomography, X-Ray ComputedABSTRACT
No disponible
No disponible
Subject(s)
Female , Adult , Humans , Analgesics, Opioid/adverse effects , Benzodiazepines/adverse effects , Heroin/adverse effects , Respiratory Insufficiency/chemically induced , Globus Pallidus/pathology , Hypoxia, Brain/etiology , Hypoxia, Brain/pathology , Magnetic Resonance Imaging , Tomography, X-Ray ComputedABSTRACT
No disponible
No disponible
Subject(s)
Female , Middle Aged , Humans , Parkinson Disease/complications , Restless Legs Syndrome/etiology , Transcutaneous Electric Nerve Stimulation/adverse effects , Parkinson Disease/therapySubject(s)
Clomiphene/adverse effects , Fertility Agents, Female/adverse effects , Migraine Disorders , Stroke/etiology , Adult , Female , Humans , Middle Aged , Migraine Disorders/chemically induced , Migraine Disorders/complications , Posterior Cerebral Artery/pathology , Pregnancy , Stroke/pathologyABSTRACT
No disponible
No disponible
