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… Expand

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References

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Neurologic involvement in Urbach‐Wiethe's disease (lipoid proteinosis)
TLDR
The clinical features consist of hoarseness, cutaneous and mucosal lesions, intracranial calcifications, alopecia, ocular and dental abnormalities, photosensitivity, and short stature. Expand
CT scan appearances in a patient with lipoid proteinosis.
TLDR
The pathogenesis is unknown; the only constant feature is deposition of an amorphous hyaline material that stains with PAS reagent in the affected tissues. Expand
Case report: computed tomography findings in lipoid proteinosis: report of two cases.
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 autosomalExpand
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.Expand
Radiological finding in lipoid proteinosis
TLDR
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. Expand
Radiological findings in lipoid proteinosis.
TLDR
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. Expand
Remarkable response of lipoid proteinosis to oral dimethyl sulphoxide
TLDR
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. Expand
Lipoid proteinosis: clinical, histologic, and ultrastructural investigations.
TLDR
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. Expand
Lipoid proteinosis presenting with neuropsychiatric manifestations.
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 calcificationExpand
Lipoid proteinosis in three children
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. AllExpand
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