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Cerebellum Science Center Cerebellar Disorders

About the cerebellum

The word “cerebellum” means “little brain” in Latin, which is fitting: while this small area at the back of the brain comprises just 10 percent of the brain’s total volume, it contains the majority of its neurons. It’s also functionally connected to every part of the nervous system, helping different brain areas work together.

Scientists once believed the cerebellum only played a role in balance and movement, but we now know it also contributes to many non-motor behaviors, like language, sleep, breathing, and regulating emotions. These discoveries have has led to greater understanding of the cerebellum’s contribution to a wide range of brain disorders and diseases. Individuals with damage to their cerebellum may have trouble with rapidly alternating movements, modulating the speed or distance of their movements, coordinating multiple body parts, and generating clear speech. They may have involuntary movements such as rhythmic movements of the eyes, called “nystagmus,” or shaking of parts of the body, called “tremor.” 

Cerebellar diseases

Diseases that affect or involve the cerebellum include:

Aging  

The cerebellum may be a central target in aging. It is well known that as we age, motor function deteriorates and in many cases tremor arises. Purkinje cells and other cerebellar cell types die as we age. As a result, falls become a leading cause of death in the elderly. There is mounting evidence to suggest that the cerebellum is central not only to these motor problems, but it may also contribute to cognitive decline with age.

Alcoholism (chronic alcohol abuse)  

The cerebellum is a primary target for alcohol. Even small amounts cause disruptions in balance, gait, reaction time, and speech. Chronic alcohol abuse causes degeneration of Purkinje cells. The symptoms are often very similar to ataxia. The sobriety test given by police officers at the roadside is in fact a test of temporary cerebellar dysfunction due to alcohol consumption above the legal limit.

Ataxia  

A type of unsteady, discoordinated movement. Ataxia can be caused by genetic and non-genetic factors. Among the most well studied are the Spinocerebellar Ataxias (SCA). Although there are many forms of ataxia as an independent disease, there are likely even more conditions that have ataxia as a core symptom.

Autism  

A spectrum of disorders impacting social, behavioral, and cognitive abilities. Both motor and non-motor concerns in autism may be linked to altered cerebellar development and cerebellar dysfunction.

Cancer  

Many types of cancer affect the cerebellum. The most common malignant pediatric brain tumor, medulloblastoma, forms in the cerebellum. Other pediatric cancers also frequently form in the cerebellum, including astrocytomas and ependymomas.

Cerebral Palsy  

Insults to the cerebellum have been associated with ataxic cerebral palsy.

Chiari Malformations  

Part of the cerebellum dips down through the bottom of the skull. This can happen due to unusual skull development or other conditions like spina bifida.

Dandy-Walker Syndrome  

A congenital alteration in the development of the cerebellum.

Dyspraxia 

(also called Developmental Coordination Disorder, DCD) is a neurological disorder characterized by the disruption of high-level integration of motor planning, execution, and action. It affects the ability of an individual to translate thoughts into actions. 

Dystonia  

Unusually timed, involuntary muscle contractions. Often expressed as co- or over-contractions that can be painful and debilitating. There are many forms of genetic dystonia, although the most common forms are non-genetic. Dystonia is the third most common movement disease.

Epilepsy  

Chronic epilepsy results in Purkinje cell loss, especially in progressive myoclonic epilepsy and Batten disease (neuronal ceroid lipofuscinosis). Alternatively, research also suggests that defective cerebellar function may also lead to seizures.

Fetal Alcohol Syndrome (FAS)  

The cerebellum is a primary target for alcohol in utero. Heavy alcohol use during pregnancy can result in early onset cerebellar degeneration. Purkinje cells are particularly sensitive although other developing cerebellar cells are also vulnerable. As a result, children are born with severe motor abnormalities and a variety of cognitive problems.

Metabolic Diseases  

The cerebellum is commonly affected in metabolic disease and often Purkinje cells degenerate. A particularly severe example is Niemann Pick Disease.

Mitochondrial Diseases  

Also referred to as Primary Mitochondrial Diseases (PMD). The cerebellum is commonly affected in mitochondrial disease and often Purkinje cells degenerate. Patients often develop ataxia and, in many cases, also epilepsy may be linked to the cerebellum circuit. POLG variants are an example.

Muscular Dystrophy  

A group of genetic conditions that cause the loss of muscle fibers leading to progressive weakness and loss of muscle use over time. It is caused by a wide variety of mutations in genes necessary for healthy muscle fiber structure and function.

Multiple Sclerosis  

The cerebellum is commonly affected in multiple sclerosis, a disease that causes degeneration of brain cells and the connections between them.

Multiple System Atrophy  

Parts of the brain, with subtypes including the cerebellum, shrink, causing wide-ranging symptoms.

Nystagmus  

An involuntary rhythmic shaking of the eyes.

Paraneoplastic Syndromes 

Cerebellar degeneration or irregular functioning of the cerebellum can occur when the body’s immune system reacts to the presence of cancer.

Prematurity 

The cerebellum is often a primary target of prematurity and related in utero complications such as infections and immune regulation.

Schizophrenia

Schizophrenia has been associated with reduced cerebellar volume and layered functional connectivity between the cerebellum and other parts of the brain.

Stroke  

A stroke occurs when there is an interruption of blood flow to part of the brain. Cerebellar strokes often cause difficulty with coordination.

Toxin and Drug Exposures 

Cerebellar cell loss has been associated with chronic alcohol abuse as well as other toxin exposures. Ongoing cerebellar activity can be widely modulated by toxin and drug exposures.

Tremor  

An involuntary rhythmic shaking of parts of the body. This is the most common movement disorder. There are many forms of tremor as an independent disease (Essential Tremor is the most common) although many other diseases also have tremor as a key symptom, such as Parkinson’s Disease.

Traumatic Brain Injury (TBI)  

Damage to the brain that is often caused by falls. Often associated with concussion, dizziness, nausea. 

Vertigo  

A sensation of motion that is often associated with dizziness and nausea.