RT Journal Article
SR Electronic
T1 CASE REPORT: PANDYSAUTONOMIA
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP e1
OP e1
DO 10.1136/jnnp-2016-315106.200
VO 87
IS 12
A1 Aseel Al-Ansari
A1 Chrispother Rickards
A1 Linzi Thomas
A1 Steve Bain
YR 2016
UL http://jnnp.bmj.com/content/87/12/e1.114.abstract
AB We present the case of a 25 year old previously healthy female who became unwell with abdominal pain and vomiting a day after taking part in a half marathon and was found to have a pseudo-obstruction requiring laparotomy. Post surgery she developed a dry mouth and urinary retention and was consistently tachycardic with labile blood pressure. She developed one episode of parotitis which resolved with antibiotics. Family history included relatives with Addison's disease, hypothyroidism and Raynaud's phenomenon.Neurological examination was normal. There were no other findings to suggest systemic autoimmune disease. Vasculitic screen was normal. Other desirable tests included porphyria screen, HIV serology and nerve conduction studies. Initial treatment with IV Methylprednisolone resulted in little improvement. IVIG and plasmapheresis likewise were not beneficial. Despite this, gradual improvement was noted over a period of several months to near normality. There have been no relapses to date.The differential diagnosis of pandysautonomia is wide and in this particular case Sjogrens disease, Guillain-Barré syndrome, porphyria, HIV infection and paraneoplastic aetiology were amongst the differentials. Autoimmune autonomic ganglionopathy was also considered given absence of evidence for other conditions, although in this case anti-ganglionic AChR antibodies were negative.