Male patient, 40 years old, a healthy daughter, with no history of morbid illness or smoking, started symptoms in 2001, with episodes of both mental disorders and difficulty in acquiring a previous bipedalism face to face in a museum pain
The motor deficit increased slowly, with a fluctuating pattern during the day, with greater weakness in the mornings.
During four years, the clinical picture a was not diagnosed spontaneously until the loss of gait, difficulty in extension of the neck, non-progressive dysphagia, dry mouth, proximal and distal muscular weakness in both extremities
Evaluated in February 2005, at the Center for Recognition of Health Dr. Allende, he observed a patient with normal auscultation in wheels, unable to incorporate, normotensive, normal lung auscultation and no other abnormalities.
In the motor exam, she presented moderate muscular weakness in thighs and cephaloparesis moderate, uniform, mild, proximal and distal paresis in the upper extremities.
In the lower limbs, he had moderate proximal and distal motor deficit, which prevented standing.
The paretic muscles presented easy fatigability and, after maximum sustained effort, a transient and brief recovery of strength.
Osteotendinous reflexes were generally decreased and facilitated after exercise.
There was no alteration of the superior functions, diplopia, dysarthria, sensory deficits, pyramidal signs or cerebellar signs.
The neurophysiological evaluation showed normal handicapped people within four limbs.
The autonomic function study showed symptoms of cholinergic dysfunction, with the presence of constipation, erectile dysfunction and increased frequency of urination.
The reflex study showed attenuation of responses.
Motor evoked potentials at rest showed decreased amplitude in all muscles explored and, after a brief maximal effort, markedly increased amplitude.
For example, the evoked motor potential of the short muscle relaxant, right thumb amplitude showed 2.7 mv at rest and after exertion, 2.9 mv at rest, and 0.6 left fifth finger at rest.
In these same muscles, a stimulating stimulation test with high frequency of stimulation, 20 hz, 30 hz and tetanization (50 Hz), showed an increase in motor evoked response amplitude greater than 1,000%.
The electromyographic evaluation showed myopic signs in the proximal muscles of the lower limbs.
A computed axial tomography of the chest and an upper endoscopy showed no pathological findings.
With these backgrounds, the clinical and neurophysiological diagnosis of idiopathic Lambert-Eaton myasthenic syndrome was made, immunologically confirmed in the Mayo Clinic laboratory with high levels of serum calcium acetmol-dependent antibodies.
Treatment was initiated with prednisone at a dose of 1.5 mg per kilogram of weight (90 mg) and neostigmine 15 mg four times a day.
After three weeks of treatment, the patient was able to stand up without support, count with 100 m before fatigue and use his upper limbs normally.
At the beginning of the fourth week of treatment, azathioprine 100 mg daily was added.
After four months, the patient recovered the self-valence, being able to obtain a kilometer before presenting signs of fatigue and ceding the symptoms of autonomic dysfunction.
The prednisone dose has decreased to 60 mg per day and azathioprine has been maintained at 100 mg per day.
