Cerebral palsy is a nonprogressive neurological disorder characterized by various cognitive, behavioral and motor disturbances that are present from early life. Ataxic cerebral palsy is one of the four subtypes in which cerebellar symptoms of impaired coordination and gait disturbances predominate. Neuroimaging procedures, primarily magnetic resonance imaging (MRI), can detect parenchymal abnormalities in the majority of cases.
Presentation
Cerebral palsy (CP) encompasses several motor, behavioral and cognitive childhood disorders of the brain that develop as a result of improper brain development or injury [1] [2]. It is a rather common clinical entity, seen in approximately 1-2 per 1000 individuals, but the risk increases up to 100 times in severely preterm newborns, thus supporting the theory of brain dysgenesis as the main factor for the appearance of CP [1]. Based on the clinical presentation, four main categories are recognized - spastic, athetoid (or dyskinetic), ataxic and mixed, with ataxic CP comprising 5-10% of cases [2] [3]. The name "ataxic" implies that the main symptoms are related to the cerebellum and its functions, such as coordination and muscle movement difficulties, as well as the inability to maintain balance and posture [2] [4]. Patients are often unable to perform the desired task, as they are unable to control the rhythm, force or accuracy of movement, particularly with fine and rapid movements [3] [4]. Furthermore, generalized hypotonia, seizures, language disorders and tremor are frequently observed in this group of individuals [2] [4]. An important feature of CP, particularly the ataxic form, is the nonprogressive nature of symptoms, which is why the term "nonprogressive cerebellar ataxia" is mentioned as a synonym in the medical literature [2].
Entire Body System
- Cerebral Palsy
We conclude that at least some subtypes of cerebral palsy may be caused by de novo genetic mutations and patients with a clinical diagnosis of cerebral palsy should be genetically investigated before causation is ascribed to perinatal asphyxia or other [ncbi.nlm.nih.gov]
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. [en.wikipedia.org]
Ataxic cerebral palsy accounts for 5–10% of all forms of CP and it is estimated that approximately 50% of ataxic cerebral palsy is inherited as an autosomal recessive trait. [doi.org]
Philadelphia Cerebral Palsy Lawyers with Over $2 Billion Awarded There are three types of cerebral palsy (CP), and ataxic cerebral palsy is the rarest. Ataxic cerebral palsy affects only 5% to 10% of people suffering from CP. [beasleyfirm.com]
- Weakness
Hypotonia may often lead to delay in diagnosis as the doctors would have to confirm if the delay in speech is due to a weak muscle tone or any other neurological condition. [speechtherapy.ygoy.com]
Learn about this topic in these articles: characteristics In cerebral palsy Ataxic cerebral palsy is a rare form of the condition that is characterized by poor coordination, muscle weakness, an unsteady gait, and difficulty performing rapid or fine movements [britannica.com]
Due to the weak muscles as a result of the disorder, patients often have a problem with sucking and swallowing which can even affect their feeding patterns. However, this improper suction or swallowing can also cause speech impediments. [ataxiccerebralpalsyhelp.com]
Fisher Archives of neurology 1978 Three stroke patients showed weakness and pyramidal signs on one side combined with a cerebellar-like ataxia on the same side… (More) [semanticscholar.org]
OTHER THREE CEREBRAL PALSY TYPES Spastic Cerebral Palsy : Resulting in stiff and weak muscles. The most common type of cerebral palsy, accounting for approximately 70% of all cases. [youhavealawyer.com]
- Falling
Says Hannah: “I don’t fall down any more, Mommy!” [theratogs.com]
People with ataxic cerebral palsy, who often struggle to maintain balance, can fall easily. Slight nudges or changes in a walking surface can also lead to a fall. [caringforspecialneedskids.com]
Because their balance is affected, the person may also fall without reason, or be unable to compensate for being accidentally bumped or for variations in the ground surfaces or an accidental mild bump from the side. [cerebralpalsy.org.au]
Legs: (lower limbs): Walking if often difficult for those with ataxic CP and they are prone to regular falling. [birthinjuryguide.org]
- Epilepsy
Where obvious supratentorial change was present, 75% had a history of epilepsy. In ataxic cerebral palsy CT findings are common, but variable. CT change correlates better with IQ and epilepsy than clinical subtype. [ncbi.nlm.nih.gov]
Walking ability related significantly to cerebral palsy types, that is, spastic unilateral, spastic bilateral, dyskinetic, and ataxic cerebral palsy, as well as to IQ level, active epilepsy, and severe visual and hearing impairment. [pediatrics.aappublications.org]
- Difficulty Walking
They can also have difficulty speaking, poor reflexes, and walking abnormalities. Ataxic cerebral palsy Ataxic CP is the least common type of CP. [healthline.com]
It usually presents itself as lack of control in the arms and legs and difficulty walking. Ataxic cerebral palsy is a medical diagnosis, and ataxia in general is usually a symptom of an underlying occurrence. [hiehelpcenter.org]
Individuals may walk with an unsteady gait and have difficulty with motions that require precise coordination, such as writing. [christopherreeve.org]
People with spastic diplegia might have difficulty walking because tight hip and leg muscles cause their legs to pull together, turn inward, and cross at the knees (also known as scissoring ). [cdc.gov]
Gastrointestinal
- Muscle Rigidity
Spastic cerebral palsy refers to a condition in which the muscle tone is increased, causing a rigid posture in one or more extremities (arm(s) or leg(s)). [medicinenet.com]
Jaw & Teeth
- Dental Caries
Obesity, less frequently than failure to thrive Gastroesophageal reflux and associated aspiration pneumonia Constipation Dental caries Dental problems also include enamel dysgenesis, malocclusion, and gingival hyperplasia. [emedicine.medscape.com]
Cardiovascular
- Hypotension
In majority of cases, during babyhood it is expressed by hypotension and motor and speaking skills delay. Gradually, ataxia becomes weaker. Speech development depends on intelligence. [questia.com]
Liver, Gall & Pancreas
- Jaundice
Signs of Ataxic CP can also include premature birth, jaundice, delayed milestones, and multiple births. Sources: http://www.ataxiccerebralpalsy.co.uk/category/ataxic-cerebral-palsy-symptoms/ http://www.breathsounds.org/rtedu/pattern.html [ataxiccerebralpalsyhelp.com]
[…] may cause ataxic cerebral palsy include (but aren't limited to): fetal oxygen insufficiency before, during or after birth genetic predisposition and birth defects infections (affecting either a newborn or a pregnant woman) medical mistakes untreated jaundice [litigatorsinc.com]
Physical brain trauma, infections in the mother, blood disease, severe jaundice, or hypoxia (lack of oxygen to the brain), can all lead to cerebral palsy. Sadly, cerebral palsy sometimes results from a physician’s preventable mistake. [brain-injury-law-center.com]
Excessive use of Pitocin to induce labor Failure to perform a C-section in the presence of fetal distress Not responding to changes in the fetal heart rate Failure to plan a C-section given the existence of risk factors Failure to timely diagnose and treat jaundice [latonalaw.com]
Neurologic
- Tremor
These tremors, often called intention tremors may become more pronounced when the individual attempts quick or precise voluntary movements, such as writing. [dc-lawyer.com]
As they attempt to reach for an object their arm develops an "intention tremor" that is very similar to a tremor or shaking you would see in an elderly individual attempting to hold a small object. [beasleyfirm.com]
Medications like primidone and benzodiazepine, while not recommended for long term use, can alleviate some of the tremor symptoms. Botox which relaxes tightened muscles has been effective in treating voice, hand and head tremors. [en.wikipedia.org]
Botox which relaxes tightened muscles has been effective in treating voice, hand and head tremors. [cerebralpalsylawdoctor.com]
They can have an "intention tremor'', the tremor gets worse as the person's hand gets closer to the object they are trying to reach. [redbridgeserc.org]
- Seizure
Learn more about cerebral palsy malpractice cases Medical Mistakes that can cause ataxic cerebral palsy Leaving a child in the birth canal too long causing a lack of oxygen to the brain Failure to recognize and treat seizures following delivery Failure [latonalaw.com]
Generalized seizures engage the entire cerebral cortex at once, while partial seizures only involve part of the cerebral cortex. Often, generalized seizures begin as partial seizures but spread throughout the brain rapidly. [medicinenet.com]
Medical advances have allowed us to better understand the importance of goal-oriented management and attention to nutrition, safe feeding, seizures, deformity, and spasticity. [doi.org]
- Quadriplegia
Many children with spastic hemiplegia or paraplegia have normal intelligence; children with spastic quadriplegia or a mixed syndrome may have severe intellectual disability. [merckmanuals.com]
Quadriplegia (Bilateral) With quadriplegia, all four limbs, the trunk and muscles that control the mouth, tongue and pharynx are involved. Thirty percent of children with Spastic Cerebral Palsy have quadriplegia. [physio-pedia.com]
Spasticity in patients with spastic quadriplegia can be more resistant even with services and orthopedic and rehabilitative intervention. [emedicine.medscape.com]
Quadriplegia – Both arms and legs are all affected. In most cases, the muscles controlling the esophagus, tongue, and mouth are also affected. Triplegia – Three limbs are affected (e.g., both legs and one arm, or one leg and both arms). [birthinjurycenter.com]
- Poor Coordination
The word "ataxic" or "ataxia" means "poor coordination / lack of muscle control." When the cerebellum-the portion of the brain responsible for balance, depth perception, and coordination-is injured, the result may be ataxic cerebral palsy. [beasleyfirm.com]
Characterised by low muscle tone and poor coordination of movement this is a relatively rare form of the condition affecting less than 10% of children with cerebral palsy. [jmw.co.uk]
Learn about this topic in these articles: characteristics In cerebral palsy Ataxic cerebral palsy is a rare form of the condition that is characterized by poor coordination, muscle weakness, an unsteady gait, and difficulty performing rapid or fine movements [britannica.com]
Ataxic cerebral palsy is characterized by poor coordination and unsteady movements Ataxic cerebral palsy affects 5 to 10 percent of individuals diagnosed with CP, research shows. It affects balance and depth perception. [elkandelk.com]
- Monoplegia
Monoplegia – One arm or one leg is affected. Quadriplegia – Both arms and legs are all affected. In most cases, the muscles controlling the esophagus, tongue, and mouth are also affected. [birthinjurycenter.com]
Hemiplegia means one side of the body is affected, diplegia is where two limbs are affected, monoplegia where one limb is affected and quadriplegia means all four limbs (and usually the whole body) are affected. [nhs.uk]
▢ Other ____________________ ▢ None Topographical Distribution Checklist Learn more » Extent: ▢ Paresis means weakened ▢ Plegia means paralyzed Prefix: ▢ Monoplegia/Monoparesis means that only one limb is affected. [cerebralpalsy.org]
Workup
A comprehensive workup is necessary for patients in whom CP is included in the differential diagnosis, as dozens of congenital and genetic disorders have similar signs and symptoms [2]. Firstly, a detailed interview with the parents regarding the appearance and progression of symptoms should be conducted, whereas a complete physical examination, particularly focused on the neurological exam and assessment of motor function, can roughly make a distinction between various types of CP and thus aid in making a presumptive diagnosis [5]. There should be a strong clinical suspicion of CP if nonprogressive generalized hypotonia and ataxic symptoms are present [3], in which case neuroimaging studies need to be performed as soon as possible. Magnetic resonance imaging (MRI) is able to detect parenchymal defects of the brain in up to 86% of ataxic CP cases, and some of the most common findings are periventricular leukomalacia (lesions of the white matter), hydrocephalus, schizencephaly (the appearance of clefts in the brain), poorly developed corpus callosum, and cerebellar abnormalities, a hallmark of ataxic CP [1] [6]. Computed tomography (CT) can also be performed as an initial method for assessment of the brain, but many studies have identified its inferiority to MRI in detecting lesions suggestive of CP [1] [5] [6].
Treatment
Treatment by such teams involves multiple approaches. [en.wikipedia.org]
BBS score before and after treatment. [scielo.br]
Treatments for Ataxic Cerebral Palsy Since ataxic cerebral palsy results in muscle problems and incoordination, physical therapy is almost always recommended as part of an overall treatment plan. [cerebralpalsyguidance.com]
However, treatments can reduce the symptoms of ataxic Cerebral Palsy and improve the patient’s functioning and quality of life. [thecplawyer.com]
Treatment There currently isn’t a cure for cerebral palsy, but the NHS explain there are treatments available to help people with the condition have an independent life. [ok.co.uk]
Prognosis
Gabriella E Molnar, Cerebral Palsy: Prognosis and How to Judge It, Pediatric Annals, 10.3928/0090-4481-19791001-08, 8, 10, (43-56), (1979). Gabriella E. Molnar and Lawrence T. [doi.org]
Prognosis Most children survive to adulthood. Severe limitations in sucking and swallowing, which may require feeding by gastrostomy tube, decrease life expectancy. [merckmanuals.com]
Prognosis With appropriate therapeutic services, patients may be able to fully integrate academically and socially. [emedicine.medscape.com]
The term cerebral palsy does not indicate the cause or prognosis of the child with cerebral palsy. There are many possible causes of cerebral palsy. [medicinenet.com]
Etiology
Computed-tomography as an adjunct in etiologic analysis of hemiplegic cerebral-palsy. 1. Children born preterm. Neuropediatrics. 1991 ;22(1): 50 - 56. [doi.org]
Etiology Etiology of cerebral palsy is multifactorial, and a specific cause is sometimes hard to establish. Prematurity, in utero disorders, neonatal encephalopathy, and kernicterus often contribute. [merckmanuals.com]
Clinically and etiologically it belongs to heterogenic group. Practically diagnosis of ataxia is made applying the method of exclusion; in this case it becomes necessary to exclude existence of slowly progressing neurodegenerative disease. [questia.com]
Epidemiologic studies suggest that prenatal factors play a predominant role in the etiology of cerebral palsy. [emedicine.medscape.com]
Epidemiology
Cerebral palsy: classification and epidemiology. Physical Medicine and Rehabilitation Clinics of North America, 20(3), 425-452. doi: 10.1016/j.pmr.2009.06.001 See abstract Paneth, N. (2008). Establishing the diagnosis of cerebral palsy. [cerebralpalsy.org.au]
Additional impairments, as well as the presence of epilepsy, correlated significantly with walking ability and, thus, the walking ability can be an indicator of total disability load. children epidemiology cerebral palsy gross motor function impairments [pediatrics.aappublications.org]
Epidemiology Unfortunately, it is difficult to access and clarify the prevalence and incidence rate of disabilities in poor-resource settings (Gladstone, 2010). [physio-pedia.com]
The epidemiology of cerebral palsy: Incidence, impairments and risk factors. Disability and Rehabilitation, 28, 183–191. doi: 10.1080/09638280500158422. PubMed CrossRef Google Scholar Østensjø, S., Carlberg, E., & Vøllestad, N. K. (2003). [doi.org]
Epidemiologic studies suggest that prenatal factors play a predominant role in the etiology of cerebral palsy. [emedicine.medscape.com]
Pathophysiology
Pathophysiology of the corticospinal system and basal ganglia in cerebral palsy Corresponding Author E-mail address: [email protected] Smith Mental Retardation and Human Development Research Center and Department of Physiology, University of Kansas Medical [doi.org]
Pathophysiology Major events in human brain development and their peak times of occurrence include the following [19] : Primary neurulation – Weeks 3–4 of gestation Prosencephalic development – Months 2–3 of gestation Neuronal proliferation – Months 3 [emedicine.medscape.com]
Prevention
At the Rasansky Law Firm, we question why these healthcare professionals failed to do everything possible to prevent your child's injuries. [texasinjuryattorney.com]
Sadly, cerebral palsy sometimes results from a physician’s preventable mistake. [brain-injury-law-center.com]
Challenges include ensuring the wellbeing of families as well as children; tackling the issues faced lifelong by people with cerebral palsy; and the continuing need for primary, secondary, and tertiary prevention of the effects of cerebral palsy on people's [doi.org]
Other causes include: High blood pressure in the mother Meningitis Bleeding on the brain Infection in baby or mother Lack of oxygen during delivery Some of these causes are more preventable than others. [oshmanlaw.com]
Where to Start You can obtain information on this topic from the Centers for Disease Control and Prevention (CDC). [rarediseases.info.nih.gov]
References
- O’Shea TM. Diagnosis, Treatment, and Prevention of Cerebral Palsy in Near-Term/Term Infants. Clin Obstet Gynecol. 2008;51(4):816-828.
- Lee RW, Poretti A, Cohen JS, et al. A Diagnostic Approach for Cerebral Palsy in the Genomic Era. Neuromolecular Med. 2014;16(4):821-844.
- Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
- Surveillance of Cerebral Palsy in Europe. Surveillance of cerebral palsy in Europe: a collaboration of cerebral palsy surveys and registers. Surveillance of Cerebral Palsy in Europe (SCPE). Dev Med Child Neurol. 2000;42(12):816-824.
- Hadders-Algra M. Early Diagnosis and Early Intervention in Cerebral Palsy. Front Neurol. 2014;5:185.
- Korzeniewski SJ, Birbeck G, DeLano MC, Potchen MJ, Paneth N. A systematic review of neuroimaging for cerebral palsy. J Child Neurol. 2008;23(2):216-227.