Ischemic Stroke and Provoked Seizure as a Manifestation of Brugmansia suaveolens (Angel's Trumpet) Acute Intoxication: A Detailed Clinical, Electroencephalogram, and Magnetic Resonance Imaging Description and 5-Month Follow-up.

Methods: We present a detailed clinical, laboratory, electroencephalogram/magnetic resonance imaging description and a 4-month follow-up of a case of stroke and provoked seizures as manifestation of angel's trumpet intoxication.

Results/discussion: A 76-year-old woman presented with stuporous state evolving in 48 hours in bilateral mydriasis, vomiting, global aphasia, confusion, and stereotyped movement. An interictal electroencephalogram, performed 72 hours later, showed frequent generalized epileptiform discharges, and a brain magnetic resonance imaging revealed 2 small subcortical lesions in the right frontal lobe on diffusion weighted imaging sequences.

Role of Niemann-Pick Type C Disease Mutations in Dementia.

Background: Several neurological and systemic diseases can cause dementia, beyond Alzheimer's disease. Rare genetic causes are often responsible for dementia with atypical features. Recently, mutations causative for Niemann-Pick type C disease (NPC) have also been implicated in neurodegenerative diseases.

Homozygous carriers of APP A713T mutation in an autosomal dominant Alzheimer disease family.

Objective: To report, for the first time, a large autosomal dominant Alzheimer disease (AD) family in which the APP A713T mutation is present in the homozygous and heterozygous state. To date, the mutation has been reported as dominant, and in the heterozygous state associated with familial AD and cerebrovascular lesions.

Methods: The family described here has been genealogically reconstructed over 6 generations dating back to the 19th century.

Novel N-terminal domain mutation in prion protein detected in 2 patients diagnosed with frontotemporal lobar degeneration syndrome.

Prion protein gene mutations have been associated with clinical pictures mimicking neurodegenerative diseases different from inherited prion diseases (IPD). We report a novel missense P39L mutation in the N-terminal domain of prion protein in 2 patients affected by frontotemporal lobar degeneration syndrome, negative for mutations in genes causative of dementia. Neither the first carrier, a 67-year-old male in which the onset was a progressive non-fluent aphasia, nor the second carrier, a 78-year-old male affected by frontotemporal dementia and parkinsonism, showed any clinical or instrumental findings suggestive of IPD.