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Effect on the Family

Living with an incurable disease


is difficult, especially when it is
someones child. It is important
to ensure that the family of the
child is well informed about the
nature and progression of the
disease, and has the
appropriate resources. Help by
connecting the family to
support groups, family
counseling, and appropriate
medical teams, which include
but are not limited genetic
counselors, neurologists, and a
pediatrician.

How You Can Help the Child in


School:
Child should have
designated support
throughout school day
Educate teachers and
children about childs
differences
Provide medication as
indicated by childs
providers
Allow child to interact
with others and be a
part of as much as
deemed appropriate
Be an informed resource

Helpful Links &


Support Groups
For Families and
Providers
National Ataxia
Foundation;
https://www.ataxia.org
National Institute of
Neurological Disorders
and Stroke;
http://www.ninds.nih.gov/
disorders/cerebellar_dege
neration/cerebellar_dege
neration.htm
Genetic and Rare
Diseases Information
Center;
https://rarediseases.info.n
ih.gov/diseases/6019/cer
ebellardegeneration/cases/2244
1

Cerebellar
Degenerati
on
Understanding
My Walk: A Guide
for Health

Brittany Molkenthin
UCONN Student Nurse

What to look for:


Signs and Symptoms
Signature characteristic: wide
unsteady, lurching gait (ataxia)
Difficulty turning
Back and forth tremor in lower
region (trunk) of body

Cerebellar Degeneration: What is it?


The cerebellum region of the brain controls the balance and muscle coordination
of the human body. When the neurons in this region deteriorate and die, the
condition is known as cerebellar degeneration. It is likely that other regions of the
brain and central nervous system could be affected due to this degeneration
process. These areas include: the brain stem, spinal cord, and cerebral cortex.

Slow and slurred speech


(dysarthria), difficulty swallowing
(dysphagia)
Repetitive uncontrolled eye
movements (nystagmus)

Causes & Theories of


Cause

Diagnosis
Suspected with signature
sign and symptoms
(ataxia)
Inherited Genetic
Mutations: Genetic
testing and counseling (if
gene is known)
Non-Genetic, NonInherited: CT Scan, MRI
MRI is most common
diagnostic tool used

Inherited genetic mutations


that alter proteins for survival
of neurons (theory)
Paraneoplastic Disorders
associated with cancer
Secondary to other
neurological diseases which
include:
o Acute and hemorrhagic
stroke
o Cerebellar cortical
atrophy
o Spinocerebellar Ataxias
ie: Friedreich ataxia
(genetic mutations,
inherited)
o Transmissible Spongiform
Encephalopathies
o Multiple Sclerosis

Treatment
For inherited cerebellar
degeneration:
o No cure
o Supportive measures
o Physical therapy,
assistance with daily
life, supportive
medications for gait
For non-inherited,
acquire cerebellar
degeneration:
o May be reversed by
treatment of
underlying cause
o For example;
treatment of cancer
Long term outlook depends
on cause

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