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Tuberous sclerosis is an autosomal dominant disorder with genetic heterogeneity; it has a wide clinical spectrum of disease. Wolff-Parkinson-White syndrome occurring in association with tuberous sclerosis is rare in adults although it has been described in children with or without rhadomyoma.1
We present a case of Wolff-Parkinson-White syndrome in a 20-year-old female with tuberous sclerosis.
Case report
A 20-year-old girl presented with two episodes of acute onset generalised tonic clonic convulsions. There was no past history of similar episodes. Her younger sister was a known case of tuberous sclerosis on antiepileptics since childhood, but the rest of her family had not been screened at the time of diagnosis.
On examination, she had facial angiofibromas. The ultrasonogram of the right kidney showed multiple small echogenic areas in upper, mid, and lower poles--the largest measuring 0.7 × 0.8 cm in the posterior aspect of mid region consistent with angiomyolipoma. The left kidney also showed tiny echogenicities in the cortex (largest measuring 0.5 cm). A small cyst was seen in the left kidney measuring 0.5 cm (cortical cyst).
An electrocardiogram (ECG) carried out showed evidence of ventricular pre-excitation (Figure 1) and an echocardiogram did not reveal any intracardiac masses. CT scan of the brain showed subependymal calcifications. On confirmation of the diagnosis of tuberous sclerosis, she was started on regular anticonvulsants.
Figure 1. Sinus rhythm, short PR interval, wide QRS complex, and delta wave
[Image omitted. See PDF]
Discussion
Tuberous sclerosis is a genetic disorder affecting cellular differentiation and proliferation, resulting in hamartoma...