Fifty years ago, the focus on the Val30Met type of the disease, in which neurologic manifestations predominate, led to the widespread notion that hereditary transthyretinrelated amyloidosis (ATTR) was essentially a neurologic disease. It is now clear that ATTR is extremely heterogeneous on both genotypic and phenotypic grounds. The clinical spectrum of the disease ranges from an almost exclusiv...

Hemolytic uremic syndrome (HUS) can be classified as typical and atypical, and the treatment recommendations currently differ between the 2 types. Eculizumab is recommended as first-line treatment for atypical HUS; however, its use in typical HUS has been controversial. We report a case of a 10-year-old male with severe neurologic impairment who was successfully treated with eculizumab, which w...

abstract: griscelli syndrome (gs) is a rare disease first described in 1978. it is inherited in autosomal recessive pattern. this disease is characterized by partial albinism, pigmentation dilution, cellular immunodeficiency, neurological involvement & uncontrolled phases of macrophage & lymphocyte activation. we report a 5 months old iranian girl presenting with silver-gray hair,eyelashes and ...

CASE DESCRIPTION A 2-year-old spayed female domestic shorthair cat was examined because of bilateral thoracic limb weakness of acute onset. CLINICAL FINDINGS Clinical signs included muscle atrophy, paresis, depressed spinal reflexes, hyperesthesia of the thoracic limbs, and reduced jaw muscle tone. Pelvic limb reflexes were normal. Results of a neurologic examination were suggestive of multif...

Seizures and neurologic involvement have been reported in patients infected with Shiga toxin (Stx) producing E. coli, and hemolytic uremic syndrome (HUS) with neurologic involvement is associated with more severe outcome. We investigated the extent of renal and neurologic damage in mice following injection of the highly potent form of Stx, Stx2a, and less potent Stx1. As observed in previous st...

A 52-year-old man presented with left hemifacial atrophy (figure 1) beginning at age 25. There were no neurologic symptoms. Neurologic examination showed no deficits, and the limbs were symmetric. Brain MRI demonstrated left cerebral hemiatrophy (figure 2) and lack of the ipsilateral soft facial tissue. Progressive facial hemiatrophy (PFH), or ParryRomberg syndrome, is a sporadic disease of unk...

PurposeTransthyretin amyloidosis (ATTR) predominantly involves the heart (ATTR-cardiac [CA]) and nerves, may involve other organs or tissues. Disease modifying therapies improve outcomes in those with ATTR-CA neurologic involvement, however their utility post transplant (HT) combined heart/liver is uncertain. We evaluated patients who underwent HT for to determine incidence of de novo developme...

BACKGROUND Most HIV infected patients will develop some sort of neurologic involvement of the disease throughout their lives, usually in advanced stages. Neurologic symptoms may occur in acute HIV infection but myelopathy in this setting is rare. Up until this date, only two cases of transverse myelitis as a manifestation of acute HIV infection have been reported in the literature. Therapeutic ...

We report early carotid involvement by retropharyngeal abscess in a 4-year-old boy. MR imaging showed enhancement of the wall and narrowing of the lumen of the internal carotid artery, which were thought to reflect spasm and/or arteritis. Prompt treatment may have prevented hemorrhagic and neurologic complications.

A patient with advanced mycosis fungoides developed neurologic and respiratory symptoms and signs following multiple courses of chemotherapy and radiotherapy. Various repeated diagnostic procedures--including cranial computerized tomography and fiberoptic bronchoscopy with transbronchial lung biopsy--failed to demonstrate an unusual involvement of the brain and lungs by mycosis fungoides. Radio...