Urbach-Wiethe Disease(Lipoid Proteinosis)

@article{Staut1998UrbachWietheDP,
  title={Urbach-Wiethe Disease(Lipoid Proteinosis)},
  author={C C Staut and Thomas P. Naidich},
  journal={Pediatric Neurosurgery},
  year={1998},
  volume={28},
  pages={212 - 214}
}
reasoning, cognitive flexibility, judgment, decision making and planning all appear to be reduced in these patients [17]. Psychomotor seizures with olfactory hallucinations, déjà vu, automatisms such as lip smacking and facial grimacing, rage attacks and paranoia have all been reported [13, 18] in patients with temporal lobe calcifications but are not seen in all patients with such calcifications [13]. Seizures rarely begin before adulthood [3]. In Heyl’s [12] series of 20 patients, 4 had… 

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References

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Lipoid proteinosis (Urbach-Wiethe disease (Urbach, E and Wiethe, C, Lipoidosis cutis mucosae, Virchows Arch. Patholog. Anat., 273, 285-319 (1929)) is a rare generalized disease with autosomal

Specific xerostomia during Urbach-Wiethe disease.

We describe the case of a 59-year-old woman who had suffered from a typical Urbach-Wiethe disease since childhood and who complains of progressive mouth and ocular dryness since the age of 54 years.

Radiological finding in lipoid proteinosis

The radiological findings in six patients suffering from lipoid proteinosis showed thickened epiglottis, swollen arytenoids and aryepiglottic folds, thickened irregular false and true vocal cords, and increased stiffness of the vocal cords.

Radiological findings in lipoid proteinosis.

The radiological findings in six patients suffering from lipoid proteinosis showed thickened epiglottis, swollen arytenoids and aryepiglottic folds, thickened irregular false and true vocal cords, and increased stiffness of the vocal cords.

Remarkable response of lipoid proteinosis to oral dimethyl sulphoxide

After 3 years of treatment of lipoid proteinosis in a 41‐year-old man using oral dimethyl sulphoxide, the patient's skin lesions, hoarseness of voice and abnormal oesophageal function improved remarkably.

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Findings in the case of a 12-year-old boy with lipoid proteinosis show a complex relationship between type IV and type III-like collagen components.

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Two patients with lipoid proteinosis are reported in whom paranoid symptoms were the presenting feature. Both had a long standing impairment of memory and bilateral medial temporal lobe calcification

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We report on three children with lipoid proteinosis, an autosomal recessive disorder characterized by the deposition of hyaline‐like material in the skin, mucous membranes and other tissues. All

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An extensive neuropsychological investigation in a patient with bilateral amygdala damage due to Urbach-Wiethe disease showed that she had normal electrodermal activity, an important finding in view of the role that has been attributed to the amygdala in the central control of autonomic responses.