CEREBELLAR SYNDROMES

Conf. dr. Rodica Blaa

 Cerebellar syndromes
(CS I

Cerebellar disturbances:
Delayed initiation and termination of motor movements.
Inability to perform continuous repetitive movements.
Errors in smoothness and direction of a movement.
The lock of coordination or synergy of movement, especially
complex movements.
The lock of motor plasticity or learning.
Postural instability.
 CS II
General rules (GR): a) if only one side of the cerebellum is
affected, the symptoms are unilateral and ipsilateral; b) lesion of the
cerebellum produce errors in the planning and execution of
movements rather than paralysis of voluntary movements; c) If
symptoms predominate in the trunk and legs, lesion is near the
midline; d) if the symptoms are more obvious in the arms, the lesion is
in the lateral hemispheres; e) the most severe disturbances are
produced by lesions in in the superior cerebellar peduncle and deep
nuclei; f) many of the symptoms of cerebellar diseases improve
gradually with the time if the underlying disease process does not
itself progress; g) almost all patients with cerebellar lesions have some
type of gait disturbances; h) speech disturbances occur with bilateral
cerebellar damage; i) signs and symptoms similar to those produced by
cerebellar lesions can appear with disorders that affects structures
adjacent to the cerebellum of affect the afferent or efferent
connections of cerebellum; j) cerebellum is responsible for monitoring
both motor and non-motor functions.
 CS III

Ataxia: a) difficulty regulating the force, range, direction, velocity,

duration and rhythm in smooth performance of voluntary acts; b)
defective timing of sequential contraction of agonist/antagonist
muscles; c) usually cerebellar ataxia persists despite cues (unlike
ataxia due to posterior column disease affecting spinal cord).
Gait ataxia: a) unsteady during ambulation; b) waking with broad-
based gait; c) lower center of gravity; d) decrease in normal, free-
flowing arm swing; e) waking heel-to toe or running the heel of
one foot dawn the shin or the other leg while standing or waking or
lying down if difficult.
Limb ataxia: a) dysmetria; b) hypermetria; c) hypometria;
d) asynergia; e) dysdiadochokinesia; f) intention tremor (kinetic
tremor).
Truncal ataxia: a) swaying of the trunk; b) staggering gait;
c) difficulty in sitting unsupported.
 CS IV

Cerebellar dysarthria: a) abnormalities in articulation and

prosody (scanning, slurring, staccato, explosive, hesitant, garbled
speech.
Oculomotor dysfunction: a) pendular nystagmus; b) gaze-
evoked nystagmus; c) upbeat nystagmus; d) rebound nystagmus;
e) optokinetic nystagmus; f) opsoclonus; g) skew deviation;
h) occular bobbing.
Muscle hypotonia: a) usually accompanies acute hemispheric
lesions and tends to decreases with time; b) more noticeable in
upper limbs and proximal muscles; c) decreased resistance to passive
stretch; d ) pendular deep tendon reflexes; e) can exacerbate the
symptoms produced by ataxia.
Macrography: a) writing may be irregular and larger than
normal
 CS V
Lack of poise: a) inability to carry out motor activities against
the force of gravity; b) particularly evident during rapid chances in
body position or direction of movement; c) unsteadiness of gait or an
inability to sit or stand without swaying or falling; d) delays in the
initiation and termination of movement; e) intentional movements,
such as grasping or pointing, may be slowed in both the building and
the relaxation of force.
Nonmotor deficits:
Cognitive and affective changes: a) impairments of set-shifting,
abstract reasoning, verbal fluency and working memory; b) visual-
spatial disorganization and impaired of visual-spatial memory;
e) dysprosodia, agrammatism and mild anomia
Emotional, personality and behavioral changes: a) anxiety,
hyperactivity, impulsiveness, irritability, dysphoria and apathy;
b) lowering of intellectual function.
 CS VI
Archicerebellar (vestibulocerebellar) syndrome.
Disorders cause disturbances of locomotion and equilibrium,
with permanent truncal and gait ataxia.
Patients with isolated flocculonodular lesions lose their ability to
stand or to walk and tend to fall even when sitting with their eyes
open.
Abnormalities of posture and station (e.g.: head tilt) and eye
movements also occur.
Eye movement disorders (nystagmus, disturbances of vestibulo-
ocular reflexes).
Tremor is not evident and muscle tone remains normal.
When the effects of gravity are reduced by the patient lying in
bed or being physically supported, movements may be completely
normal.
 CS VII

Paleocerebellar (spinocerebellar) syndrome.

The cardinal feature is involvement of legs.
the gait is wide-based and ataxic, with small hesitant steps.
Muscles hypotonia.
Spinocerebellar ataxia reflects a more general deficit in the
control of the muscles of ambulation, where vestibulocerebellar
ataxia reflects a particular inability to control the leg muscles in
the presence of force of gravity.
 CS VIII
Neocerebellar (cerebrocerebellar) syndrome.
Damage of the lateral cerebellar hemispheres and dentate
nucleus disturbs skilled coordinated movements and speech.
Errors in direction, deviation from proper course, dysmetria, dys-
diadochokinesia and intention tremor all may be present,
especially in movements of upper extremities.
The gait may actually be normal, reflecting the relative sparing of
the axial muscles and lower limbs.
Intentional movements, such grasping or pointing, may be
delayed in their initiation and slower in both the buildup and
relaxion of intended force.
Stretch reflexes and muscle tone are often diminished, resulting
in flabbiness, lock of check and pendular deep tendon reflexes.
Dysarthric speech may occur with bilateral involvement.
Oculomotor disturbances may also occur.
 CS IX
Pancerebellar syndrome.
Bilateral signs of combination of all cerebellar syndromes.
Etiology.
Inherited: a)spinocerebellar ataxia Friedreichs ataxia;
b) cerebellar ataxia olivopontocerebellar atrophy)
Developmental: a) Arnold-Chiari malformation; b) Dandy-Walker
malformation; c) congenital cerebellar hypoplasia.
Nutritional disorders: a) Vitamin B1, B12 or E deficiency.
Neoplasic disorders: a) astrocytoma; b) medulloblastoma;
c) cerebellar metastasis; d) tumors of the cerebellopontine angle;
e) paraneoplastic cerebellar degeneration.
Demyelinating disorders: a) multiple sclerosis.
Vascular disorders: a) cerebellar infarction or hemorrhage.
Cerebral trauma in the area of the occiput.
Intoxications: a) drug-induced; b) alcohol; c) volatile solvents.