ataxia dr. shamekh m. el-shamy. the cerebellum anatomy: the cerebellum is formed of 2 main parts:...

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ATAXIA ATAXIA Dr. Shamekh M. El-Shamy Dr. Shamekh M. El-Shamy

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Page 1: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

ATAXIAATAXIA

Dr. Shamekh M. El-ShamyDr. Shamekh M. El-Shamy

Page 2: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

THE CEREBELLUMTHE CEREBELLUM ANATOMYANATOMY: : the cerebellum is the cerebellum is

formed of 2 main parts:formed of 2 main parts: 1. A midline central structure, 1. A midline central structure,

known as the vermis. known as the vermis. 2. Two lateral cerebellar 2. Two lateral cerebellar

hemispheres.hemispheres.**cerebellum can be divided:cerebellum can be divided:1- Focculo-nodular lobe.1- Focculo-nodular lobe. 2. 2. Anterior lobe.Anterior lobe. 3- 3- Posterior lobe.Posterior lobe.

Page 3: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Anterior lobe

Posterior lobe

Foocculo-nodular lobe

Paleocerebellum

Neocerebellum

Archicerebellum

Page 4: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Archicerebellunm (flocculo-Archicerebellunm (flocculo-nodular lobe):nodular lobe):

Page 5: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Paleo-cerehellum (anterior Paleo-cerehellum (anterior lobe):lobe):

Page 6: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Neocerebellum (posterior Neocerebellum (posterior lobe): lobe):

Page 7: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Blood SBlood Supplyupply

The cerebellum is supplied byThe cerebellum is supplied by:: Superior cerebellar artery Superior cerebellar artery

(branch of the basilar artery).(branch of the basilar artery). Anterior inferior cerebellar Anterior inferior cerebellar

artery artery (branch of the basilar (branch of the basilar artery). artery).

Posterior inferior cerebellar Posterior inferior cerebellar artery artery (branch of the vertebral (branch of the vertebral artery).artery).

Page 8: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Cerebellum SyndromesCerebellum Syndromes

I.I. Archicerebellar Archicerebellar syndromesyndrome: :

Page 9: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

II.II. Neo-cerebellar syndromeNeo-cerebellar syndrome::

There is incoordination of voluntary motor There is incoordination of voluntary motor activitiesactivities

Page 10: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

ATAXIAATAXIA

Definition:Definition: Incoordination of Incoordination of voluntary motor activity with or voluntary motor activity with or without disequilibration in the without disequilibration in the absence of motor weakness.absence of motor weakness.

Page 11: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Cerebellum AtaxiaCerebellum Ataxia

Causes of Cerebellar AtaxiaCauses of Cerebellar Ataxia:: I.I. Herido-familial:Herido-familial: 1. Friedreich's ataxia. 2. 1. Friedreich's ataxia. 2.

Marie's ataxia.Marie's ataxia. II. Symptomatic :II. Symptomatic : CongenitalCongenital InfectiveInfective VascularVascular ToxicToxic

Page 12: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

5. Neoplastic5. Neoplastic

6. Demyelination6. Demyelination

7. Metabolic7. Metabolic

III. Idiopathic:III. Idiopathic:

Page 13: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Clinical picture of Cerebellar Clinical picture of Cerebellar AtaxiaAtaxia

I. Incoordination of movements of I. Incoordination of movements of different muscles in the form of:different muscles in the form of:

a)Nystagmus in the eyes.a)Nystagmus in the eyes.

b) Staccato speech.b) Staccato speech.

c) Nodding of the head.c) Nodding of the head.

d) Titubaiion of the trunk.d) Titubaiion of the trunk.

e) Kinetic tremors of the e) Kinetic tremors of the limbs.limbs.

Page 14: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

II. Hypotonia and hyporeflexia II. Hypotonia and hyporeflexia of the affected muscles.of the affected muscles.

III. III. Gait disturbance: Wide Gait disturbance: Wide base or drunken gait in base or drunken gait in archicerebellar lesions.archicerebellar lesions.

IV. Positive tests: used by the IV. Positive tests: used by the neurologist to detect neurologist to detect cerebellar ataxia.cerebellar ataxia.

Page 15: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

TESTS OF CEREBELLAR TESTS OF CEREBELLAR ATAXIAATAXIA

1. 1. Finger to nose testFinger to nose test: :

Page 16: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

2. Finger to doctor's finger 2. Finger to doctor's finger test: test:

you may found you may found

a) Decomposition of a) Decomposition of movement. movement.

b) Kinetic intention tremors b) Kinetic intention tremors

c) Dysmetria in the form of c) Dysmetria in the form of hypermetria or hypometriahypermetria or hypometria

Page 17: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

3. Adiadokokinesis 3. Adiadokokinesis

Page 18: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

4. Rebound phenomenon:4. Rebound phenomenon:

Page 19: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

5. Heel to knee test5. Heel to knee test

Page 20: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

HERIPO-FAMILIAL HERIPO-FAMILIAL ATAXIASATAXIAS

I. Friedreich's AtaxiaI. Friedreich's Ataxia1. It occurs in the 1st decade of 1. It occurs in the 1st decade of

life.life.

2. Gradual onset and slowly 2. Gradual onset and slowly progressive course.progressive course.

3. Positive family history is 3. Positive family history is common as it is inherited as an common as it is inherited as an autosomal recessive disease.autosomal recessive disease.

Page 21: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

44. Pathologically there is . Pathologically there is degeneration of:degeneration of:

- Cerebellum specially the - Cerebellum specially the archicercbcllum &archicercbcllum & the the spinocerebellar tracts.spinocerebellar tracts.

- Pyramidal tracts.- Pyramidal tracts.

- Posterior columns.- Posterior columns.

- Peripheral nerves.- Peripheral nerves.P.N

Page 22: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

II. II. Marie's AtaxiaMarie's Ataxia1. It occurs in the 2nd and 3rd 1. It occurs in the 2nd and 3rd

decades of life.decades of life.

2. Gradual onset and slowly 2. Gradual onset and slowly progressive course.progressive course.

3. Pathologically there is degeneration 3. Pathologically there is degeneration of: of: 

- Cerebellum specially the neo-- Cerebellum specially the neo-cerebellum (dentate nucleus)cerebellum (dentate nucleus)

-Pyramidal tracts.-Pyramidal tracts.

Page 23: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

- It presents with progressive - It presents with progressive manifestations of neocerebellar manifestations of neocerebellar degenerationdegeneration..

- Mental impairment, ocular nerve - Mental impairment, ocular nerve paralysis and extrapyramidal paralysis and extrapyramidal syndromes are occasionally syndromes are occasionally present.present.

Page 24: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

SENSORY ATAXIASENSORY ATAXIA  

Definition:- Definition:- It is ataxia due to It is ataxia due to loss of the proprioceptive (deep) loss of the proprioceptive (deep) sensations, at any point in their sensations, at any point in their pathway.pathway.

Page 25: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Causes :-Causes :-

4. Medial lemniscus : 4. Medial lemniscus : brain brain stem lesions. stem lesions.

5. Thalamus : 5. Thalamus : thalamic thalamic syndrome. syndrome.

6. Cortical sensory area : 6. Cortical sensory area : parietal lobe lesions.parietal lobe lesions.

Page 26: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Rhomberg’s Test :Rhomberg’s Test :

Page 27: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

VESTIBULAR ATAXIAVESTIBULAR ATAXIA

Definition :- Definition :- It is ataxia due to It is ataxia due to lesions of the vestibular division of lesions of the vestibular division of the eighth nerve.the eighth nerve.

Page 28: ATAXIA Dr. Shamekh M. El-Shamy. THE CEREBELLUM ANATOMY: the cerebellum is formed of 2 main parts: ANATOMY: the cerebellum is formed of 2 main parts: 1

Thank you