Cerebral Palsy in the 21<sup>st</sup> Century: Cerebellar Injury in the Survivors of Extreme Prematurity

Special Article – Cerebral Palsy

Phys Med Rehabil Int. 2016; 3(3): 1086.

Cerebral Palsy in the 21st Century: Cerebellar Injury in the Survivors of Extreme Prematurity

Bodensteiner JB*

Department of Neurology and Pediatrics, Mayo Clinic, 200 First Street SW, Rochester, MN, USA

*Corresponding author: John B. Bodensteiner, Department of Neurology and Pediatrics, Mayo Clinic, 200 First Street SW, Rochester, MN, USA

Received: April 12, 2016; Accepted: June 21, 2016; Published: June 23, 2016


The face of cerebral palsy is changing in the 21st century. Infants who are born extremely prematurely (Less than 1Kg and Less than 28 weeks of gestation) are surviving in increasing numbers, both with and without brain injury. The incidence of cerebral palsy is between 10 and 15% in these patients. As a result they account for an increasing proportion of patients with cerebral palsy. In this paper I review the classical forms of CP as defined by neurological criteria and then discuss some of the more or less unique features of cerebral palsy in the survivors of extreme premature birth.

Keywords: Cerebral Palsy; Extreme prematurity; Cerebellar injury; Pseudobulbar affect


Cerebral palsy has been around as long as Homo sapiens. The definition of the condition we call Cerebral Palsy (CP) varies widely among health care providers depending on the orientation of the individual provider involved. For some, the presence of a nonprogressive dysfunction of the central nervous system that affects the motor function of the individual is sufficient for the diagnosis. I believe, however, that a more discriminating definition is useful if the physician is to draw conclusions about the etiology, management and prognosis of the patient and his/her motor disability. To state it differently, from the standpoint of the neurologist, the term CP should be used when the injury which results in motor disability is stable and non-progressive, occurs at or near the time of birth, involves a previously normal and obviously immature nervous system, and affects the motor system (at least). In this definition the etiology and the timing of the brain injury is important. This definition also excludes malformations of the brain from the diagnosis of CP which may at first seem inappropriate but if a diagnosis is to allow one to understand something about the etiology, infer something about the clinical features to be expected, and to some extent, the prognosis and management going forward, then a more exacting definition of the condition is necessary. In the practice of medicine these days, a diagnosis has become just a tool for billing purposes but the true meaning of a medical diagnosis is more than that.

Once the patient has been determined to have CP there are as many different classifications of the disability as there are different health care providers involved in their care. One classification would describe the nature of the motor disability such as spastic, dystonic or ataxic. Another would describe the distribution of the disability such as diplegia, quadriplegia, hemiplegia, bilateral hemiplegia, appendicular or truncal or gait ataxia. Functional classifications can be useful also to indicate the severity of the disability. Most use a combination of various classifications such as Mild spastic hemiplegia or moderate spastic diplegia etc.

Classical Forms of Cerebral Palsy

The most common types of CP are fairly clearly delineated and can usually be correlated with the nature of the insult and the timing of the insult. I will describe the typical clinical types, their neuropathological correlates and the timing and nature of the brain insult which gives rise to the type of CP.

Spastic CP

It is useful to further divide spastic CP into descriptions of the distribution of the motor involvement, for example: Spastic diplegia, spastic tetraplegia (spastic quadriplegia), spastic hemiplegia and spastic double hemiplegia. Each subtype typically has its own clinical history with respect to the timing and nature of the insult, and its own prognosis. Each of the subtypes of spastic CP will also have distinct implications for management and outcome of the patients.

Spastic Tetraplegia

Spastic tetraplegia (spastic quadriplegia) involves all four limbs more or less equally. The typical story is that this is the result of a catastrophic event near the end of the pregnancy (Close to term) where there is loss of blood flow and global asphyxia. The infant is comatose for several days and requires ventilation and blood pressure support for several days as well. The most common causal events would include placental abruption or uterine rupture or maternal shock. The clinical result is a microcephalic child with global disabilities including microcephaly and intellectual disabilities. The neuropathological spasticity and correlate of this type of CP is called multicystic encephalomalacia (Figure 1) and the imaging may also show hydrocephalus ex vacuo (Figure 2).