Cerebellar syndrome - Symptoms, Causes and Treatments

Definition

A syndrome cerebellar is a disorder, the origin of which is linked to an attack on a part of the cerebellum, or anatomical areas connecting this organ to the rest of the central nervous system.

Symptoms

Symptoms of cerebellar syndrome include:

Problems with standing.
Gait disturbances.
L'adiadochokinesis of Babinski (term derived from à: privative, diadokhos: succeeding, and kinêsis: movement), corresponds to the disappearance of diadochokinesis, which is the capacity to be able to quickly carry out certain movements such as:
- Pronation (taking an object).
- Supination (extending the hand with the palm facing upwards to beg) and this in an alternating manner. Adiadochokinesia is a symptom observed in a patient with a lesion of the cerebellum. This condition is also found in other pathologies of the nervous system such as multiple sclerosis.
It could also be a asynergy, that is to say that complex movements are broken down into several stages:
- So if a patient is asked to lift his foot, he will do this in several steps.
- When the patient is standing, he tends to spread his feet to maintain his balance and increase his base of support (surface delimited on the ground and corresponding to the space necessary for a person to maintain his balance).
- The patient has oscillations that do not worsen when the eyes are closed. These are of large amplitude and irregular.
- The patient's gait resembles that of a drunken man (we speak of drunken gait), with a tendency to deviate towards the side of the lesion.
- The movements begin with a certain delay, and most often exceed the patient's goal. Direction is nevertheless preserved, and there is sometimes a tremor.
- Tremors, on the other hand, are not present at rest. They only appear during voluntary movements made by the patient.
- Tendency to fall when standing. When the subject is affected on only one side of the cerebellum (unilateral cerebellar syndrome), it tends to deviate to the side of the lesion in most cases.
- When the cerebellar syndrome is bilateral, the subject sways but does not fall.
- Speech disorders: the emission of words is jerky, there is a separate pronunciation of syllables, diction is slow, the patient hesitates. In some of them, the tendency is to speak explosively with grimaces. This is linked to a disturbance in the coordination of the muscles allowing phonation and articulation of speech. This syndrome is observed mainly in bilateral cerebellar syndrome.
- When the central part of the cerebellum is affected, it is mainly the standing position that is affected, as well as walking. In the case of lesions of the lateral parts, it is movement that is impaired.

physiology

Le cerebellum being one of the regulatory centers of themotor cerebral cortex (at the origin of the movements), the symptoms presented by the patient are different depending on the injured area:

The achievement of wyrm leads to the occurrence of static balance disorders (when the patient remains motionless): we then speak ofataxia cerebellar.
The achievement of hemispheres mainly causes movement coordination disorders.
Involvement of the posterior part of the cerebellum causes a nystagmus (involuntary low-amplitude oscillating and rotating movements of the eyeball) among others.

Pathophysiology

Le Claude syndrome (in English Claude's syndrome), is secondary to a lesion of an area of the cerebellum: the cerebral peduncle. This set of symptoms is characterized by:

A speech difficulty (problems speaking).
Paralysis of certain ocular nerves (the common ocular motor nerve) on the side of the lesion, the patient presents with what some call a cerebellar hemiplegia (non-significant term) actually one cerebellar hemi-syndrome. This is a set of symptoms corresponding to the lesion of half of the cerebellum. This type of condition is mainly encountered when a particular area of the cerebellum is affected: the red core (more precisely, we are talking about alternating red nucleus syndrome).

La Fahr's disease (in English Fahr's disease) is a condition whose causes are poorly understood. For some specialists, it is a disruption in the use of calcium in the body. Indeed, there is an impairment of the central gray nuclei (islands of gray cells within the white matter of the cerebellum), associated with deposits of calcium carbonate, which gradually penetrate these areas. This pathology, which affects men in middle age, is also characterized by an attack on the dentate nuclei of the cerebellum which makes the lesion visible on skull X-rays. The symptoms presented by the patient are:

Slowness and rarity of movement, also called akinesia.
A hypertonia, that is to say, rigidity and trembling of the limbs at rest indicating the presence of Parkinsonian syndrome. pyramidal bundle is a main nerve pathway, belonging to the central nervous system (brain and spinal cord). It is made up of a group of nerve fibers, having a common path, and intended to carry voluntary motor messages (nerve impulses allowing movement to be obtained, unlike messages intended for the perception of sensations). It connects the pyramidal-shaped nerve cells contained in the cerebral cortex (gray matter of the brain) to other nerve cells contained in the spinal cord.
A epilepsy.
A cerebellar syndrome.
A change in character.
Intellectual disorders appearing gradually.
In some patients, insufficient secretion of parathyroid hormone. parathyroid glands supervise the maintenance of calcemia (quantity of calcium in the blood), while calcitonin, a hormone secreted by the thyroid, has the opposite role, that is to say, to lower the level of calcium in the blood. The evolution of this pathology is pejorative. Indeed, it leads to the death of the patient within a few years.

La Kuru disease, fatal, affecting the cerebellum and causing symptoms similar to those of Parkinson's disease (tremors, walking difficulties, movement coordination problems, dementia). This is the first condition for which the prions (responsible, in particular, for the Creutzfeldt-Jacob disease). Kuru once affected the Papuan tribes of New Guinea. It was caused by the ingestion of contaminated brains: in fact, these tribes had adopted the habit of eating the brains of the dead to pay them homage. The epidemic died out with the suppression of cannibalism.

La Strümpell Lorrain disease is a neurological condition characterized by spastic paraplegia (paralysis of the lower limbs), due to a demyelination (progressive destruction of myelin = fatty sheath surrounding certain nerves). This demyelination affects certain areas of the central nervous system which are the pyramidal tracts, and sometimes (milder damage) the spinocerebellar tract. Strümpell-Lorrain syndrome includes early forms which appear at the age of 2 or 3 years, and late forms, which sometimes only appear after 35 years.

Le Seemann syndrome corresponds to a disorder of the development of the cerebellum accompanied by incoordination of movements without impairment of muscular strength, due to damage to the central nervous system. Added to this is a delay in the onset of speech, dizziness, and nystagmus.

Le Arns-Sayre syndrome, also called Kearns-Sayre syndrome, or Barnard-Scholz syndrome, corresponds to a set of symptoms due to a disruption in the use of DNA from mitochondria, which are small organs contained in the cytoplasm of cells, and having the shape of small spheres, or rods, playing an important role in the management of the body's energy. The patient affected by this pathology presents:

An inflammation of the retina (called pigmentary).
External ophthalmoplegia (disturbances in the movement of the eye muscles) with ptosis (drooping of the upper eyelids).
Divergent strabismus (lack of parallelism of the eyes).
Damage to the myocardium (heart muscle) with disturbances in the conduction of nerve impulses inside the heart, leading to changes in its beating (bundle branch block, atrioventricular block).
Most often, patients also present hearing problems such as deafness.
A cerebellar syndrome.
A disruption in the secretion of hormones (endocrine disorders).
A size below average.

La Hunt's disease or syndrome (in English: Ramsay Hunt's syndrome), also called facial or auricular shingles, is characterized by the presence of spontaneous pain, or provoked (by an injury or irritation of infectious origin), located on the path of a nerve, at the level of its roots (which connect it to the central nervous system), or in the area that it innervates. This pain presents a paroxysm (episode during which the symptoms manifest themselves with the greatest intensity). During this pathology, patients complain of pain in the ear canal and the middle ear.

Le Sicard's cerebello-occipito-vertebral angle syndrome, also called Sicard syndrome, is secondary to a lesion of a part of the nervous system (the medulla oblongata), and is characterized by the presence of paralysis of the sternocleidomastoid muscle and the trapezius, anesthesia of the anatomical territories innervated by the first two nerves from the cervical spine (vertebrae of the neck), and a cerebellar syndrome.

Le lateral bulbar syndrome corresponds to a set of symptoms generally due to a progressive destruction in the form of softening on the side of the medulla oblongata, the origin of which is a disorder of the blood supply by disturbance of the circulation. This syndrome is characterized by the presence of hemiplegia (paralysis of half the body) which alternates, and a cerebellar hemisyndrome. Some patients present with paralysis of the soft palate of the pharynx and the larynx.

Pontine syndrome, also called mesocephalic or pontine syndrome, is a syndrome secondary to damage to the pons, or mesocephalon, or Varole's bridge, that is to say the part of the brainstem located above the bulb, below the cerebral peduncles, and in front of the cerebellum.

L'cerebellar hemiplegia (non-significant term) is in fact a cerebellar hemisyndrome. It is a set of symptoms corresponding to the lesion of half of the cerebellum. This type of lesion is mainly found when a particular area of the cerebellum is affected: the red core (more precisely, we are talking about alternating red nucleus syndrome).

Le superior cerebellar artery syndrome causes voluntary movements, tremors, and disturbances in the perception of heat and pain in patients. Astrocytoma is a relatively benign tumor of the central nervous system (cerebellum, brain, spinal cord more rarely), which tends to encyst. This type of tumor develops at the expense of astrocytes.

Medical exam

Physical examination

The limb on the affected side falls without the patient being able to hold it inertly when the examiner lifts this limb and suddenly lets go.

Le Holmes rebound phenomenon, is highlighted in some cases. When the examiner tries to extend the patient's forearm against his will (i.e. against his resistance), and suddenly releases this forearm, the patient's hand rebounds (in a way) towards the shoulder.

Cause

The conditions that cause cerebellar syndrome affect the cerebellum, just one of its components, or the fibers that provide connection between this organ and other parts of the nervous system.

It could be:

In affection reaching the vessels (hemorrhage, softening).
In infection (abscess, cerebellar encephalitis, syphilis).
In intoxication (drugs, alcohol).
In some patients, cerebellar involvement is of paraneoplastic origin, leading to subacute cerebellar degeneration. It may also be primary cerebellar atrophy (olivopontocerebellar atrophy, cerebellar cortex atrophy).
Finally, cerebellar disease can be of hereditary and familial origin: this is the case, among others, of Friedrich's disease and Pierre Marie's heredo-cerebellar ataxia.