Cerebellar System And Ataxia

Question 1

Give an example of heridetary cerebellar ataxia with autosomal dominant inheritance?

Answer 1

Spinocerebellar ataxia SCA

Question 2

What are the five hereditary cerebellar ataxia is with autosomal recessive inheritance

Answer 2

1)friedreich’s ataxia
2)ataxia-telangiectasia
3) Ataxia associated with vitamin E deficiency
4) Ataxia oculomotor apraxia
5) Ataxia associated with coenzyme Q 10 deficiency 

Question 3

Which genetic mutation is involved in friedreich’s ataxia?

Answer 3

GAA trinucleotide repeat in frataxin gene

Question 4

What is the mortality cause in Friedreich’s ataxia?

Answer 4

Cardiac involvement

Question 5

What are the clinical findings observed in Friedreich’s ataxia?

Answer 5

Scoliosis, pes cavus, cardiomyopathy, diabetes myelitis and deafness

Question 6

What is the most common recessive ataxia accompanied with sensory findings and a reflexia due to degeneration of the posterior cord and dorsal root ganglia on?

Answer 6

Friedreich’s ataxia

Question 7

What is the definitive diagnosis in Friedreich’s ataxia

Answer 7

Genetic testing

Question 8

What are the causes of acquired ataxia with chronic course?

Answer 8

1) Alcoholic cerebellar degeneration
2) Vitamin E deficiency
3) New variant Creutzfeldt-Jakob disease 
4) Chronic inflammatory demyelinating polyneuropathy (CIDP)

Question 9

What are the causes of acquired ataxia with acute or subacute onset?

Question 10

What is dysarthria

Answer 10

Synergy between muscles that provide speech function is impaired; pt speaks as if drunk

Question 11

What is tremor what does it occur asa result from?

Answer 11

In cerebellum diseases, it occurs during coordinated movement

Question 12

What is ataxia

Question 13

What is hypotonia in cerebellum lesion

Answer 13

Loss of tonus affecting ipsilateral side of cerebellar hemisphere

Question 14

Ataxia in midline lesions of the cerebellum is referred to as what?

Answer 14

Trunk ataxia in which the patient has difficulty understanding and working

Question 15

Is hypotonia answerable and lesions ipsilateral or contralateral?

Answer 15

Hypotonia is observed in the arm and leg on the same (ipse lateral) of affected cerebellar hemisphere

Question 16

What is sensory ataxia

Question 17

The …….. is associated with the ipsilateral side of the body
,While the ……….. is associated with the contralateral side

Answer 17

1-cerebellum
2-cerebral cortex

Question 18

Vertigo, nystagmus, and ataxia occur in lesions of which part of the CNS?

Answer 18

In lesions of vestibular part of cerebellum (archi-cerebellum)

Question 19

What are the clinical findings in cerebellum lesions?

Answer 19

Ataxia(sensory or vestibular)
Tremor
Dysmertia
Dysarthria
Hypotonia
Dyssynergia
Dysdiadokinesia

Question 20

Which of the following has extremity involvement ; hemispheric, vestibular or vermis?

Question 21

Sensory ataxia occurs due to which lesion?

Question 22

**Where is the lesion observed in vestibular ataxia?

Answer 22

In Nucleus vestibularis and its central connections

Question 23

1+ Lesions in the ………produce limb (appendicular) ataxia.
2+Lesions of the ……. cause truncal and gait ataxia (NOT affecting the limbs, & NO dysmertia and Dysdiadokinesia.)
3+…… lesions cause disequilibrium, vertigo, and gait ataxia.

Answer 23

1+CEREBELLAR HEMISPHERE
2+ VERMIS[mid-line]**
3+VESTIBULOCEREBELLAR