Effects of age and cognition on a cross-cultural paediatric adaptation of the Sniffin' Sticks Identification Test.

Objectives: To study the effects of age and cognition on the performance of children aged 3 to 18 years on a culturally adapted version of the 16 item smell identification test from Sniffin' Sticks (SS16).

Methods: A series of pilots were conducted on 29 children aged 3 to 18 years old and 23 adults to produce an adapted version of the SS16 suitable for Brazilian children (SS16-Child). A final version was applied to 51 children alongside a picture identification test (PIT-SS16-Child) to access cognitive abilities involved in the smell identification task.

Clinical Findings and Natural History in Ten Unrelated Families with Juvenile and Adult GM1 Gangliosidosis.

We describe 12 subjects of ten unrelated families from the region of Campinas and the southern state of Minas Gerais, Brazil, who presented with juvenile (n = 4) and adult (n = 8) GM1 gangliosidosis. Data includes clinical history, physical examination, and ancillary exam findings. Six subjects presented initially with skeletal deformities, while the remaining six had neurological manifestations at onset.