Progressive sensory neuropathy in children

[Progressive, sensory neuropathy in 2 children].

Hereditary sensory neuropathy type

Hereditary sensory neuropathy type I (HSN I) is a slowly progressive neurological disorder characterised by prominent predominantly distal sensory loss, autonomic disturbances, autosomal dominant inheritance, and juvenile or adulthood disease onset. The exact prevalence is unknown, but is estimated as very low. Disease onset varies between the 2nd and 5th decade of life. The main clinical featu...

Congenital sensory neuropathy.

Progressive auditory neuropathy in patients with Leber's hereditary optic neuropathy.

OBJECTIVE To investigate auditory neural involvement in patients with Leber's hereditary optic neuropathy (LHON). METHODS Auditory assessment was undertaken in two patients with LHON. One was a 45 year old woman with Harding disease (multiple-sclerosis-like illness and positive 11778mtDNA mutation) and mild auditory symptoms, whose auditory function was monitored over five years. The other wa...

Hereditary sensory and autonomic neuropathy: A case report

A 24-year old female patient with the history of pressure ulcers in distal extremities resulted in severe deformity will be reported. Her disease started when she was 9 years old and a similar history was found in her brother. In physical examination, pain and temperature sensations were impaired in distal extremities. Nerve conduction velocity showed impaired sensory and normal motor responses...