[Ptosis and mastication disorders revealing concurrent myasthenia gravis and chronic polyradiculoneuritis].
Although myasthenia gravis (MG) has frequently been associated with other autoimmune disorders, it has only rarely been reported in conjunction with diseases of the nervous system. A 74-year-old patient with hypertension suddenly presented left unilateral ptosis and mastication disorders. Clinical examination showed a concomitant loss of strength distally and reduced deep tendon reflex. Electrophysiologic data indicated a diagnosis of MG and chronic inflammatory demyelinating polyneuropathy; acetylcholine receptor antibody was elevated at 4.1 nmol/L (normal < 2 nmol/L). Improvement was rapid after initiation of pyridostigmine in association with corticosteroid (1 mg/kg/day). One month later, the cranial nerve deficit disappeared and strength was normal. It is likely that a basic abnormality of immune regulation was responsible for the emergence of diseases with different clinical presentations, but similar immunopathogenesis. Corticosteroid seemed to be the most effective treatment.
Magot
A
A
Clinique Neurologique, CHU Nantes.
Wiertlewski
S
S
Boutoleau
C
C
Guiheneuc
P
P
Fève
J R
JR
Damier
P
P
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D001763:Blepharoptosis / Q000188:drug therapy / Q000209:etiology*; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use; D003389:Cranial Nerve Diseases / Q000188:drug therapy / Q000503:physiopathology; D003711:Demyelinating Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D004594:Electrophysiology; D001068:Feeding and Eating Disorders / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D008297:Male; D008409:Mastication / Q000502:physiology*; D009157:Myasthenia Gravis / Q000150:complications* / Q000175:diagnosis; D011115:Polyneuropathies / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011729:Pyridostigmine Bromide / Q000627:therapeutic use; D011950:Receptors, Cholinergic / Q000378:metabolism; D012026:Reflex, Stretch / Q000502:physiology
D000305:Adrenal Cortex Hormones; D002800:Cholinesterase Inhibitors; D011950:Receptors, Cholinergic; D011729:Pyridostigmine Bromide