Spontaneous intracerebral hemorrhage in Urbach-Wiethe disease

Urbach-Wiethe syndrome

“…Complications encountered in these patients include dental hypoplasia, gum hypertrophy, dryness of mouth, recurrent parotid and submandibular gland infections, and predisposition to respiratory distress during an episode of upper respiratory infection, due to thickened tongue and larynx 5 6. Neurological complications include basal ganglia calcification, usually bilaterally symmetric, which can predispose to seizures, subtle cognitive abnormalities7 and, rarely, spontaneous intracerebral haemorrhage 8. Usually, patients have a normal lifespan.…”

Spontaneous intracerebral hemorrhage in Urbach-Wiethe disease

“…Helio A. Teive, Curitiba; Eduardo Ruschel, Joinville; Renato P. Munhoz, Curitiba, Brazil: Messina et al 1 presented a 39-year-old woman with Urbach-Wiethe disease or lipoid proteinosis (LP) who developed right hemiparesis due to a left lenticular nucleus hemorrhage. The authors suggested that LP is associated with diffuse small-vessel disease.…”

“…This abnormality could explain the susceptibility of brain blood vessel rupture. 1 We published a case report of generalized dystonia and striatal calcifications in a patient with LP. 3 Family history was positive for a 34-year-old sister diagnosed with LP who had epilepsy and mild mental retardation.…”

“…Steven R. Brenner, St. Louis: While this image is beautiful, 1 it seems to have gone beyond the intended purpose of the original research on the mice. I understand publishing the original mouse brain image based on the striking images as an educational process and raising awareness of the experimental animals, technology, and the findings involved.…”

“…2 Author Response: Fernando Calamante, Melbourne: I appreciate the comment by Dr. Brenner regarding the brain kaleidoscope image. 1 Further to his comment, we would like to clarify that the image used to construct the kaleidoscope was from a dataset acquired for justifiable scientific purposes. 3 The subsequent use of the image for artistic expression should not detract from the primary purpose of these data.…”