Ataxia Flashcards
Frederich’s Ataxia:
Autosomal recessive - trinucleotide repeat
What reflexes are positive for Frederich’s? What is negative?
Romberg and Babinski, absent DTRS and vibration/position
What is Frederich’s Ataxia Physiology?
Spinal cord degeneration - lose spinocerebellar and posterior column
Diagnostics for Fredreich’s (2):
Gene testing, nerve conduction
What is the onset and progression of Frederich’s Ataxia?
Onset at age 20 or before
- Ataxia
- Later speech
- UE extremities
Ataxia Telengiectasia?
Autosomal recessive
Pathology of ataxia telangiectasia?
Demyelination of nerves and cerebellar degeneration
Diagnostics for Ataxia telangiectasia?
Serum alpha fetoprotein
Carcinoembryonic antigen
Onset of ataxia telangiectasia?
Infancy - progressive - cannot walk by age 12
What is a clinical feature of ataxia telangiectasia in the eyes?
Telangiectasia in the eye
What is immune mediated ataxia?
Purkinje cells in the cerebellum are cross reacted with the body cells (gluten, paraneoplastic etc.)
What are three infectious causes of ataxia?
- Cerebellar abscess (through the ear)
- Brainstem encephalitis (listeria, lyme, prion)
- Meningitis
Post infectious ADEM:
Acute disseminated encephalomyelitis - demyelinating of the brain and the spinal cord
Treatment for ADEM:
IV methylprednisolone and IVIG
Most common cause of ataxia in children:
Acute cerebellar ataxia
