[Pure subacute pandysautonomia: an assessment of treatment with intravenous polyvalent immunoglobulins].
BACKGROUND
Acute or sub-acute pure dysautonomia is uncommon. We report a case of sub-acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy.


METHODS
A 29-year-old right-handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea, vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l) was the only cerebrospinal fluid anomaly. Serum tests were negative for anti-gangliosides antibodies. The patient improved slowly after two series of intravenous immunoglobulin infusions.


CONCLUSIONS
Clinical course and laboratory findings suggest that acute or sub-acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. Therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.
Ramirez
C
C
Service de Neurologie D, Hôpital R. Salengro, CHRU, 59037 Lille Cedex.
de Sèze
J
J
Stojkovic
T
T
Ferriby
D
D
Delalande
S
S
Defoort-Dhellemmes
S
S
Vermersch
P
P
D000208:Acute Disease; D000328:Adult; D001342:Autonomic Nervous System Diseases / Q000150:complications / Q000628:therapy*; D005759:Gastroenteritis / Q000209:etiology; D006801:Humans; D007024:Hypotension, Orthostatic / Q000209:etiology; D007116:Immunization, Passive; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D009886:Ophthalmoplegia / Q000209:etiology; D011128:Polyradiculopathy / Q000209:etiology / Q000628:therapy; D014786:Vision Disorders / Q000209:etiology; D014987:Xerostomia
D016756:Immunoglobulins, Intravenous