Acute renal failure due to hyperhidrosis secondary to Shapiro's syndrome

Author: V. Dimov, M.D., Department of General Internal Medicine, Cleveland Clinic

A 55 yo CM was transferred to our hospital with ARF diagnosed at an 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.

PMH:
AFib, DVT, obesity, mild mental retardation, HTN, GERD

Medications:
Coumadin, Cardizem, Nexium

FMH:
No family history of hyperhidrosis

ROS:
Episodic drenching whole body sweats, fatigue, mild decrease in appetite, no weight changes

Physical examination:
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

Laboratory results:


Severe ARF in Shapiro's syndrome which resolved completely with IVF

What happened?
The patient continued to have episodes of rigors and profound drenching sweats 4 times daily. HIV antibody was negative. CT 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.

Conclusion:
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. on 2/9/2007. 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

Final diagnosis:
Acute renal failure due to hyperhidrosis secondary to Shapiro's syndrome

References:
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.


Created: 04/13/2007