52-year-old male with a history of splenectomy at the age of 8 years due to possible B-thalassaemia and no cardiovascular risk factors. Smoker of 1 pack/day and moderate drinker. Allergic to pyrazolones. Risky sexual relations in his youth.
He was admitted for presenting, coinciding with a chronic dental infection, headache with oral discomfort for a week, to which had been added 48 hours earlier facial paralysis, initially on the left, of sudden onset, which after 24 hours became bilateral with no other accompanying symptoms.
General examination revealed angioma on the forehead and right eye, together with bilateral parotid swelling. Initial neurological examination revealed bilateral peripheral facial paralysis causing dysarthria with bilateral Bell's sign. Tearing and hearing were not affected. Taste alteration in the anterior third of the tongue. During the days following admission, paraesthesia was added to the fingers, initially in the right upper limb and later bilaterally, and loss of strength in the 4 limbs, predominantly proximal, with paresis 4/5 in the right upper limb and 4+/5 in the left upper limb on neurological examination, together with bilateral tricipital and right stylo-radial arreflexia. The clinical picture continued to progress with the addition of paresis in the lower extremities 4/5 of the bilateral hip flexors of right predominance with abolished osteotendinous patellar and Achilles reflexes. Independent gait with clumsiness, preserving tiptoe and heel gait but with weakness in both quadriceps. He had a normal chest X-ray with normal blood count, biochemistry and coagulation. Serology (Borrelia, Brucella, toxoplasma, rubella, lupus, EBV, CMV, HSV, HIV, Varicella-Zoster, measles and mumps) in blood and CSF were negative. Blood RCT and immunoglobulin quantification were normal. Neuroimaging tests, CT and brain MRI, showed no findings of interest. CSF showed increased protein (1.5 gr/dl), no leukocytes and positive pandy with negative cytology. No oligoclonal bands were observed. Tensilon test was negative. ENG was performed on the seventh day of admission, where absence of bilateral peripheral facial response was observed with signs of sensory-motor polyneuropathy in the extremities, subsequently repeated after 14 days, persisting motor conduction disturbances of demyelinating type with severe bilateral facial involvement, proximal-distal in the upper extremities and proximal predominance in the lower extremities. Initially, treatment with corticoids was started with no clear clinical response and, given the progressive evolution of the condition, treatment was started with immunoglobulins i.v. for 5 days (28 g/day), with no further progression of the symptoms and with the onset of clinical remission mainly in the extremities and very discreetly of the facial paralysis that persisted at the time of discharge. After six months, the patient has shown complete improvement in both the facial paralysis and the weakness in the limbs and does not require further rehabilitation treatment.