Cerebellar ataxia
Clinic
- It is a form of ataxia originating in the cerebellum.
- Non-progressive congenital ataxia (NPCA) is a classical presentation of cerebral ataxias.
- 33% of people with isolated, late onset cerebellar ataxia go on to develop multiple system atrophy.
- Cerebellum's role is not purely motor. It is combined with intellect, emotion and planning (Cerebellar cognitive affective syndrome)
Sign / Symptoms
- Ataxia, Incoordination of balance, Gait, extremity and Eye movements
- Dyssynergia, Dysmetria, dysdiadochokinesia
- Dysarthria: Slurring of speech, tremor of the voice and ataxic respiration
- Asthenia, Asynergy, Delayed reaction time
- Dyschronometria
- Dysphagia
- Hypotonia
- Ipsilateral movements deficits
- Nystagmus
- Tremor of head and trunk (titubation)
Causes
- Gluten ataxia
- CNS vasculitis
- Infarction, tumors, direct injury, toxins, genetic disorders
Types
Cerebellar ataxias can be classified as
- Sporadic
- Autosomal recessive
- X-linked, autosomal dominant
- Mitochondrial origin
Other types are
- Post viral cerebellar ataxia = Acute cerebellitis = Acute cerebellar ataxia
- Primary auto-immune ataxias (PACA)
Related disease
- Opsoclonus Myoclonus syndrome is an inflammatory disease which involves Cerebellum
- MS
Miasms
Dysfunction of the cerebrocerebellum
(Lateral hemispheres) Appendicular ataxia |
Intention tremor
Coarse trembling Accentuated over the execution of voluntary movements Possibly involving head, eyes, limbs and torso |
Peculiar writing abnormalities
Large, unequal letters Irregular underlining |
Peculiar Dysarthria
Slurred speech characterized by explosive variations in voice intensity despite a regular rhythm |
Dysdiadochokinesia
Inability to perform rapidly alternating movements which could involve rapidly switching from pronation to supination of the forearm. Movements become more irregular with increases of speed. |
Dysmetria
Hypometria or overshooting Hypermetria
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Rebound phenomenon
Loss of the check reflex |
Schmahmann's syndrome
Patients may exhibit a constellation of subtle to overt cognitive symptoms |
Dysfunction of the spinocerebellum
(Vermis and areas near the midline) |
Truncal ataxia:
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Vestibulocerebellum dysfunction
Flocculonodular lobe |
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