Cerebellar disorders have numerous causes, incl. Congenital malformations, hereditary ataxias and acquired disorders. Although the symptoms vary depending on the cause, but this is typically associated ataxia (impaired muscle coordination). The diagnosis is made clinically, often on the basis of an imaging and sometimes due to genetic testing. Treatment is usually supportive, unless the cause is acquired and reversible.
The cerebellum consists of 3 parts:
Cerebellar disorders have numerous causes, incl. Congenital malformations, hereditary ataxias and acquired disorders. Although the symptoms vary depending on the cause, but this is typically associated ataxia (impaired muscle coordination). The diagnosis is made clinically, often on the basis of an imaging and sometimes due to genetic testing. Treatment is usually supportive, unless the cause is acquired and reversible. The cerebellum consists of 3 parts: archicerebellum (vestibulocerebellum): It includes the flocculonodular lobe, which is located in the medial zone. The archicerebellum is involved in the maintenance of balance and coordination of eye, head and neck movements; it is closely connected with the vestibular nuclei. Median situated worm (vermis, Paläocerebellum): It helps to coordinate the torso and leg movements. Lesions of the worm lead to abnormalities of stance and gait. Lateral hemispheres (neocerebellum): control fast and feinkoordinierte movements of the extremities, v. a. the poor. The consensus is growing that in addition to coordinating the cerebellum also controls some aspects of memory, learning and cognition. Ataxia is the archetypal signs of cerebellar dysfunction, but also many other motor abnormalities can occur (see table: characters cerebellar disorders). Sign cerebellar disorders deficit manifestation ataxia tumbling, broadbased transitional unloads inability fine to take coordinated actions in the right order dysarthria inability to articulate words correctly, with vague and inappropriate language Dysdiadochokinesie inability to quickly perform alternating movements dysmetria inability to control the range of movement hypotension Reduced muscle tone nystagmus involuntary, rapid oscillation of the eyeballs in a horizontal, vertical or rotational direction, the speed component maximum in the direction of the side of the Kleinhirnläsion comprises scanning speech Slow articulation with a tendency at the beginning of hesitation word or syllable Tremor Rhythmic, alternating, oscillating movement of a limb, once a target approaches (intentional tremor), or proximal muscle when a fixed posture or the lifting of a weight is attempted (postural tremor) etiology Congenital malformations such malformations are almost always sporadically before, they often appear as part of a complex malformation syndromes (eg. As Dandy-Walker syndrome), which betriffen other parts of the brain. Abnormalities manifest early in life and are not progressive. The manifestations vary significantly depending on the affected structures; ataxia is usually vor.Hereditäre ataxia Hereditary ataxias may be autosomal recessive or autosomal dominant trait. Autosomal recessive ataxias include Friedreich’s ataxia (is most common), the ataxia telangiectasia with that Abetalipoproteinemia (Bassen-grain branch syndrome), ataxia with isolated vitamin E deficiency and the Cerebrotendinous cholesterosis. The Friedreich’s ataxia due to a genetic mutation that causes abnormal repeats of the DNA sequence of GAA in the gene encoding the mitochondrial protein frataxin. Inheritance occurs as an autosomal recessive. Decreased Frataxinspiegel lead to mitochondrial iron overload and impaired mitochondrial function. When Friedreich’s ataxia is unsteadiness between 5 and 15 years of age is noticeable; followed by an ataxia of the upper extremities, dysarthria and paresis, v. a. the lower extremities. The intellectual abilities allow for frequent. A tremor is, if any, easily. The reflections and the vibration and position sense are lost. Often talipes equinovarus (clubfoot), scoliosis and progressive cardiomyopathy. At the end of the third decade of life, patients can be confined to a wheelchair. The death, often due to cardiac arrhythmia or heart failure, usually occurs in middle age. Spinocerebellar ataxias (SCA) are the most common autosomal dominant ataxias. The classification of these ataxias has recently been revised several times since the knowledge of genetics increases. Currently, at least 28 different loci are known; about 10 thereof include the expanded DNA repeats. Some of these involve repeats of the DNA sequence CAG coding for the amino acid glutamine, similar to Huntington’s disease. The symptoms of SCAs varied. Some of the most verbreitenen SCA relate to various areas in the central and peripheral nervous system: often have neuropathy, pyramidal signs and restless legs syndrome and ataxia. Some SCA only cause cerebellar ataxia usually. SCA type 3, formerly known as Machado-Joseph disease, the most common dominantly inherited SCA may be worldwide. Symptoms include ataxia, Parkinsonism and possibly dystonia, facial twitching, ophthalmoplegia and peculiar bulging Augen.Erworbene disorders Acquired ataxia can occur as a result of nichthereditären neurodegenerative diseases (eg. As Multsystematrophie), systemic diseases, multiple sclerosis, cerebellar infarctions, repeated traumatic brain injury, toxin exposure or it may be idiopathic. Systemic diseases include alcoholism (alcohol toxic cerebellar degeneration), celiac disease, heat stroke, hyperthyroidism and vitamin E deficiency. Among the toxins include carbon monoxide, heavy metals, lithium, phenytoin and certain solvents. Toxic concentrations of certain drugs (eg. As anti-epileptic drugs) can cause cerebellar disorders and ataxia. In children, a primary brain tumor (medulloblastoma, cystic astrocytoma) may be the cause; the center line of the cerebellum is the most common location of such a tumor. Rarely reversible diffuse cerebellar dysfunction develop in children as a result of a viral type infection. Diagnosis Clinical Investigation Typically MRI Sometimes genetic testing The diagnosis of cerebral diseases is made clinically and includes a thorough family history and search for acquired systemic diseases. It will be (typically an MRT) conducted a neural imaging. Based on advice from a family history of genetic testing is performed. Treatment Treatment of the cause, if possible Normally, only supportive Some systemic diseases (. Eg hypothyroidism, celiac disease) and Toxin exposure may be treated; occasionally, a surgical intervention with structural lesions (tumors, hydrocephalus) is useful. Usually, however, the treatment is only supportive.