Ataxic Cerebral Palsy

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.

This disorder originates from congenital processes.

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].

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

Prognosis

Etiology

Epidemiology

Sex distribution
Age distribution

Pathophysiology

Prevention

Summary

Patient Information

Search symptoms now!

References

  1. O’Shea TM. Diagnosis, Treatment, and Prevention of Cerebral Palsy in Near-Term/Term Infants. Clin Obstet Gynecol. 2008;51(4):816-828.
  2. 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.
  3. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
  4. 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.
  5. Hadders-Algra M. Early Diagnosis and Early Intervention in Cerebral Palsy. Front Neurol. 2014;5:185.
  6. 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.

  • Cerebral palsy-definition, classification, etiology and early diagnosis - C Sankar, N Mundkur - Indian journal of Pediatrics, 2005 - Springer
  • A neurophysiological basis for the treatment of cerebral palsy - K Bobath - 1991 - books.google.com
  • A neurophysiological basis for the treatment of cerebral palsy - K Bobath - 1991 - books.google.com
  • Cerebral palsy epidemiology: where are we now and where are we going? - L Mutch, E Alberman, B Hagberg - Medicine & Child , 1992 - Wiley Online Library
  • Cerebral palsy: an overview - KW Krigger - American family physician, 2006 - media.kenanaonline.com
  • A gene for ataxic cerebral palsy maps to chromosome 9p12-q12. - DP McHale, AP Jackson, MI Levene, P Corry - European journal of , 2000 - ukpmc.ac.uk
  • A survey of 400 cases of cerebral palsy in childhood - P Asher, FE Schonell - Archives of disease in childhood, 1950 - adc.bmj.com
  • A report: the definition and classification of cerebral palsy April 2006 - P Rosenbaum, N Paneth, A Leviton - Dev Med Child , 2007 - Wiley Online Library
  • Ataxia telangiectasia - HL Utian, M Plit - Journal of Neurology, Neurosurgery, and , 1964 - ncbi.nlm.nih.gov
  • Ataxia-telangiectasia - E Boder, RP Sedgwick - Pediatrics, 1958 - Am Acad Pediatrics
Search symptoms now!