Ataxic cerebral palsy – Wikipedia, the free encyclopedia

Ataxic cerebral palsy is clinically observed in approximately 5-10% of all cases of cerebral palsy, making it the least frequent form of cerebral palsy diagnosed.[1]Ataxic cerebral palsy is caused by damage to cerebellar structures, differentiating it from the other two forms of cerebral palsy, which are spastic cerebral palsy (damage to cortical motor areas and underlying white matter) and athetoid cerebral palsy (damage to basal ganglia).[2]

Because of the damage to the cerebellum, which is essential for coordinating muscle movements and balance, patients with ataxic cerebral palsy experience problems in coordination, specifically in their arms, legs, and trunk. Ataxic cerebral palsy is known to decrease muscle tone.[3]

The most common manifestation of ataxic cerebral palsy is intention (action) tremor, which is especially apparent when carrying out precise movements, such as tying shoe laces or writing with a pencil. This symptom gets progressively worse as the movement persists, causing the hand to shake. As the hand gets closer to accomplishing the intended task, the trembling intensifies which makes it even more difficult to complete.[4]

Like all forms of CP, there is no "cure for" ataxic cerebral palsy. However there are a number of diverse treatments which together have been used to limit the negative effects of the condition. Like all forms of CP it is most common for ataxic cerebral palsy to be congenital, resulting from errors in the development of the cerebellum and connexins during pregnancy. However it is also possible to be acquired via meningitis or even by head trauma.,[5] although the latter more often leads to one of the many forms of traumatic brain injury, which is categorically separate from cerebral palsy as a class.

The first documented clinical interest in childhood cerebellar disorders was seen in 1861 when Friedreich published his paper titled familial spino-cerebellar degeneration. His further studies of the late 19th century noted symptoms including tremor, hypotonia, diminished or lost tendon jerks, and slurred speech. It was in 1897 when Freud, having studied Friedreichs work and other research of the time, suggested that there be a special category of cerebral palsy called ataxic cerebral palsy.[5]

Approximately 2-2.5% of children born in the western world have cerebral palsy, with increasing incidence in twin and premature births.[6] Ataxic cerebral palsy accounts for 5 to 10% of all cases.[7] The cause of cerebral palsy, in particular its ataxic subtype is unknown, but thought to be due to malformation or damage in the cerebellum and its many connections,.[5][7] The majority of cases that present malformation of the cerebellum are congenital, however acquired ataxic cerebral palsy can result from meningitis, trauma, birth complications, and encephalopathies (septic, acute, disseminated, and toxic).[5] In addition, maternal viral infections may cause damage to the fetal brain due to increase in inflammatory cytokines produced during infection. Brain injury can occur during prenatal, perinatal, or postnatal periods. Most cases of cerebral palsy, approximately 80%, are acquired prenatally from unknown causes. Incidence increases with decreasing gestational periodfewer than 32 weeks of gestation and birth weight less than 5 Ib 8 Oz or 2500g.[7]

Due to impaired balance, patients suffering from ataxic cerebral palsy often walk with their feet unusually far apart (a wide gait). In addition, the low muscle tone caused by ataxic cerebral palsy causes people suffering from the disease to appear very unsteady, as their body is constantly trying to counterbalance itself. Infants with the disease often take a significantly longer amount of time to be able to walk without support, and over 50% of all children with ataxic cerebral palsy experience some form of a learning disability or speech impediment.[1]

The condition, whether resulting from cerebellar malformation or injury, results in incomplete cerebral development and no two people are affected in the same way.[6] In general, cerebral palsy is a physical impairment that affects posture and the development of movement. Ataxic cerebral palsy in particular, is manifested in the performance of movements with abnormal force, rhythm, and accuracy.[4] Patients have hypotonia (decreased muscle tone), signs of ataxia (loss of full control of bodily movement), impaired balance and coordination, intention tremors, and a wide-based gait (in walking patients).[7]

Cerebral development normally occurs in the first two years of life when the infant is acquiring new motor and adaptive skills, consequently signs and symptoms of ataxic cerebral palsy begin to manifest during this time period. Typically patients fail to reach motor milestones and show a qualitative difference in motor development.[6] During the neonatal period (first 28 days of life), children are noted to be lethargic, relatively immobile, and floppy.[5] Moreover, hypotonia is greatest during this period, even though muscle tone increases with age, it never reaches normal levels.[5] The limbs show weakness, incoordination in voluntary movement, dysdiadochokinesis (in inability to perform rapidly alternating movements), and titubation.[5]

Diagnosis of ataxic cerebral palsy is based on clinical assessment using standardized assessment tools. Diagnosis begins with the observation of slow motor development, abnormal muscle tone, and unusual posture in children that fail to reach developmental milestones.[7] Diagnosis differs in adults and children because a childs brain is still developing and acquiring new motor, linguistic, adaptive, and social skills.[5] The testing strategy is based on the pattern of development of symptoms, the patients family history, and any factors that might influence the diagnosis, such as injury or trauma.[7] Associated disabilities such as the those previously described under symptoms associated with ataxic cerebral palsy, i.e., sensory impairment and cognitive dysfunction, are also helpful in diagnosing the disease.

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Ataxic cerebral palsy - Wikipedia, the free encyclopedia