Cerebellum Pathology Flashcards
what is arnold-chiari malformation
-a group of congenital hindbrain malformations affecting the cerebellum, brainstem and spinal cord
• Both sexes affected; onset usually dependent on type
• Types- 4
• Etiology unknown
what are the different types of arnold-chiari malforation
• Type 1- herniation of cerebellar tonsils not medulla through FM. Only one
that can be acquired (infection, injury, exposure to toxic substances)
• TYPE 2- usually ass. With Spina Bifida. Classic ACM- both brainstem and
cerebellum herniates thru FM
• TYPEs 3 & 4 rare and incompatible with life
what are the symptoms of arnold-chiari malformation
•Hydrocephalus (non-communicating)
•Headache (due to increased intracranial pressure)
•Cerebellar ataxia
oLack of coordination, paralysis of extremities, cerebellar ataxia, sensory disorders
•Mental dullness
•Visual disturbance, diplopia
•Myelomeningocele (Type II)
what is medulloblastoma
- obstructs flow of CFS from the cerebral aquiduct through the 4th ventricle – Epidemiology: most often in children (median age of 2years) – Males>females
what are the impairments associated with medulloblastoma
• hydrocephalus • truncal ataxia • ataxic, staggering gait • little or no limb ataxia • sometimes spontaneous nystagmus • sometimes rotated postures of head= compressing of CN • signs of raised intracranial pressure
what is the pathology of arnold-chiari malformation
• Genetic defect causes compression and herniation of the cerebellum as it grows
• Results in 3 pathophysiological consequences:
o Compression of the medulla and upper spinal cord
o Compression of the cerebellum
o Disruption of CSF through the foramen magnum hydrocephalus
what is friedreich’s ataxia
an autosomal recessive degenerative disease of the cerebellum, lateral column and dorsal column of the spinal cord
what structures are involved in friedreich’s ataxia
- Cerebellum
- Dorsal column system (sensory neurons of fasciculus gracilis and cuneatus)
- Corticospinal tract (motor neurons)
what is the pathology of friedreich’s ataxia
LOOK AT NOTES PG 78-79
What are S&S of friedreich’s ataxia
Neurological
oCerebellar ataxia (uncoordinated movement, dysmetria, disdiadochokinesia, dysarthria, nystagmus, intention tremor, decomposition of movement, pendula reflexes)
oSensory ataxia (loss of balance from lack of proprioceptive info)
oMuscle weakness
oPeripheral neuropathy
Other
oScoliosis (may impair breathing), pes cavus
oCardiomyopathy
oDiabetes (due to low exercise ability)
what is the clinical course of friedriech’s ataxia
- Symptoms usually appear between the ages of 5 and 15 (can appear as early as 18months, late as 30 years) – the individual must have developed movement for symptoms to show
- First symptom = usually difficult walking, clubfoot, flexion of toes (due to high spasticity), hammer toes, foot inversion
- Nystagmus is common
- Gradually worsens and spreads to the arms, then trunk
- Progresses to wheelchair dependent by the early thirties
- Death is common from heart failure
what are some impairments of someone with friedreich’s ataxia
LOOK AT NOTES PG 79
what is ataxia
loss of coordination of the muscles, especially the extremities. Occurs when there is a lesion to the cerebellum
what is dysarthria
difficult or unclear articulation of speech that is otherwise linguistically normal
what is nystagmus
an involuntary, rapid and repetitive movement of the eyes
