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

• 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

MMP

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 Inability to judge distances or ranges of movement happens.	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: Uncertain starts and stops Lateral deviations Unequal steps
Vestibulocerebellum dysfunction Flocculonodular lobe	Balance disturbances Control of eye movements Postural instability Negative Romberg's test