Author: V. Dimov, M.D., Assistant Professor, University of Chicago
Reviewer: S. Randhawa, M.D.
A 55-year-old Caucasian male (CM) was transferred to our hospital with acute renal failure (ARF) diagnosed at an outside hospital (OSH) where he was admitted for generalized weakness and chills. He has had episodes of total body severe diaphoresis for the past 1.5 months.
Past medical history (PMH)
Atrial fibrillation (AFib), deep vein thrombosis (DVT), obesity, mild mental retardation, hypertension (HTN), GERD.
Warfarin (Coumadin), Cardizem (diltiazem), Nexium (esomeprazole).
Family medical history (FMH)
No family history of hyperhidrosis.
Review of systems (ROS)
Episodic drenching whole body sweats, fatigue, mild decrease in appetite, no weight changes.
VS 35.6-102-20-164/118, 98% on 2 L nasal cannula.
A morbidly obese man with flat affect shivering under 4 blankets which are wet presumably from prior episode of diaphoresis.
Skin exam: all skin surfaces wet.
Cardiovascular: Irregularly irregular rhythm, tachycardic.
Lungs: Clear to auscultation bilaterally.
Abdomen: Soft, nontender, and obese.
Extremities: No edema.
Severe ARF in Shapiro's syndrome which resolved completely with intravenous fluids (IVF).
The patient continued to have episodes of rigors and profound drenching sweats 4 times daily. HIV antibody was negative. CT of the chest showed borderline cardiomegaly, pulmonary arterial hypertension, and no nodules or consolidations. MRI of the abdomen showed no adenopathy, normal liver/spleen/pancreas/kidneys/GB, and no adrenal masses.
MRI of the brain showed findings compatible with agenesis of the corpus callosum.
This morbidly obese man with mild mental retardation who was previously healthy until this year presents with severe episodes of hyperhidrosis and chills. It is interesting that he also also has agenesis of the corpus callosum discovered on MRI of the brain after admission. This constellation of findings is consistent with a clinical picture of Shapiro's syndrome.
Shapiro's syndrome is diagnosed by the combination of hypothermia, severe hyperhidrosis, subtotal agenesis of the corpus callosum, onset typically in adulthood.
Treatment of hyperhidrosis in the the above settings could be achieved with cyproheptadine, clonidine or glycopyrrolate. Topiramate can also be used.
What happened next?
The patient was started on topiramate with good effect. He had one more episode of ARF which was milder and resolved rapidly with IVF. At the last follow-up appointment, one month after discharge, hyperhidrosis has been satisfactory controlled with topiramate.
Mild ARF in Shapiro's syndrome which resolved over 24 hours with IVF.
Serum creatinine shows episodes of ARF in Shapiro's syndrome.
Acute renal failure due to hyperhidrosis secondary to Shapiro's syndrome.
Agenesis of the corpus callosum associated with paroxysmal hypothermia: Shapiro's syndrome. Neth J Med. 1997 Jan;50(1):29-35.
Clonidine therapy for Shapiro's syndrome. Q J Med. 1992 Mar;82(299):235-45.
Mayo Clinic, April 20, 2011: Hyperhidrosis is a condition in which a person sweats uncontrollably. There are many treatment options for hyperhidrosis and in some cases surgery may be needed. Dawn E. Jaroszewski, M.D., a cardiothoracic surgeon, at Mayo Clinic in Arizona, explains one of the surgical treatment options -- a minimally invasive procedure called sympathectomy. Please note: This video contains graphic images of a surgical procedure.