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Hydranencephaly and Cerebral Palsy

Posted Oct 02 2009 3:07pm
I remember one of the first bits of information I found in the beginning on theRays of Sunshineweb page, one of the many that stayed in my mind over the past year, was that hydranencephaly would be easier to explain by just saying that Brayden has Cerebral Palsy ( CP ). I never researched how much truth was in this statement until the last week or so, I'd never actually known how broad of a condition CP was until now. And it does, now, make sense how it would indeed be easier to say my son has CP rather than giving a long and detailed explanation into the condition of hydranencephaly. It's sad, that so many rare conditions such as hydranencephaly exist that are so easily classified into another group and forgotten about as an individual condition...maybe for hydran that will change one day!

The term cerebral palsy refers to any one of a number of neurological disorders that appear in infancy or early childhood and permanently affect body movement and muscle coordination but don’t worsen over time. Even though cerebral palsy affects muscle movement, it isn ’t caused by problems in the muscles or nerves. It is caused by abnormalities in parts of the brain that control muscle movements. The majority of children with cerebral palsy are born with it, although it may not be detected until months or years later. The early signs of cerebral palsy usually appear before a child reaches 3 years of age. The most common are a lack of muscle coordination when performing voluntary movements (ataxia); stiff or tight muscles and exaggerated reflexes ( spasticity ); walking with one foot or leg dragging; walking on the toes, a crouched gait, or a “scissored” gait; and muscle tone that is either too stiff or too floppy. A small number of children have cerebral palsy as the result of brain damage in the first few months or years of life, brain infections such as bacterial meningitis or viral encephalitis, or head injury from a motor vehicle accident, a fall, or child abuse.

Since ultimately, a child with hydranencephaly can be looked at as having a traumatic brain injury (and many times has been shown to have had a stroke or other injury in- utero )...I can definitely see how it would be easier to compare. The main difference, and it's a big one, is that most children with CP don't suffer a terminal prognosis and many barely show lasting disabilities with early intervention and treatment.

There are, however, MANY similarities.

Spasticity is associated with many conditions to include CP, Hydran, Multiple Sclerosis (MS), spinal cord injuries, brain trauma or severe head injury, some metabolic diseases, and any other neuromuscular disorders. It is a condition affecting the central nervous system, in which certain muscles are continuously contracted causing stiffness/tightness and making it very difficult to move or speak, and sometimes even breath. There are varying degrees of spasticity, some like Brayden just have mild muscle stiffness, but others suffer more severely with uncontrollable, and often painful muscle spasms. Although automatically associated with these many conditions, it can be detected with the existence of increased muscle tone ( hypertonicity ), many series' of quick muscle contractions ( clonus ), stiff hard to move joints, exaggerated reflexes, etc. Many of these symptoms can be treated with daily stretches and movement...but ultimately must be treated with medications, and in some instances even surgery.

Ataxia, the lack of coordination of gross motor skills, is also shown to be found in both CP & Hydran, among other conditions...although there are 3 types of ataxia, I think the one most often found in children with hydranencephaly would be Cereballar Ataxia due to the small or total non-existence of the cerebellum. Here's the formal definition of this type fromWikipedia:

The termcerebellar ataxiais employed to indicate ataxia due to dysfunction of thecerebellum. This causes a variety of elementary neurological deficits, such as antagonisthypotonia,asynergy,dysmetria,dyschronometria, anddysdiadochokinesia. How and where these abnormalities manifest depend on which cerebellar structures are lesioned, and whether the lesion is bilateral or unilateral.

  • Vestibulo -cerebellar dysfunctionis related to the flocculonodular lobe ( flocculus and nodulus ) and involves problems regulating balance and controlling eye movements. This presents withpostural instability, in which the person tends to separate the feet on standing to gain a wider base, and avoid oscillations (especially posterior-anterior ones); instability is therefore worsened when standing with the feet together (irrespective of whether the eyes are open or closed: this is a negativeRomberg'stest).
  • Spino -cerebellar dysfunctionthe most serious of all forms, corresponds to the vermis and paravermis and presents with a wide-based "drunken sailor" gait, characterised by uncertain start and stop, lateral deviations, and unequal steps. This part of the cerebellum regulates body and limb movements.
  • Cerebro -cerebellar dysfunctionindicates a lesion of the deep pontine nuclei connections with the cerebellum which coordinates planning and monitoring of movements and presents with disturbances in carrying out voluntary, planned movements, includingintention tremor(coarse trembling, accentuated over the execution of voluntary movements, possibly involving the head and eyes as well as the limbs and torso), peculiar writing abnormalities (large, unequal letters, irregular underlining), and a peculiar pattern ofdysarthria(slurred speech, sometimes characterised by explosive variations in voice intensity despite a regular rhythm)

There are also, I have found, many types of which one hydran children are most similarly compared to would depend on the child. The types are classified as to what parts of the body are affected, and in what ways they are affected. Here are the basic forms of CP from one of my favorite websites, 4MyChildHelp and Hope for Life:

Spastic Cerebral Palsy

Spastic Cerebral Palsy is the most common diagnosis. If your child’sCPis “spastic,” her muscles are rigid and jerky, and she has difficulty getting around. There are three types of spastic Cerebral Palsy:

  • Spasticdiplegia— Your child’s leg and hip muscles are tight, and his legs cross at the knees, making it difficult to walk. This kind of movement is frequently referred to as “scissoring.”
  • Spastichemiplegia— Only one side of your child’s body is stiff. Her arms or hands might be more affected than her legs. On the affected side, her arm and leg may not develop normally. She may also require leg braces.
  • Spastic quadriplegia— The severest of the three, spastic quadriplegia means that your child is more likely to have mental retardation if diagnosed as quadriplegia. His legs, arms, and body are affected. It will be difficult for him to walk and talk, and he may also experience seizures.

AthetoidDyskineticCerebral Palsy

Athetoiddyskineticis the second most frequently diagnosed type of Cerebral Palsy. Your child will have normal intelligence, but her body will be totally affected by muscle problems. Her muscle tone can be weak or tight, and she might have trouble walking, sitting, or speaking clearly. She may also have trouble controlling her facial muscles and therefore drool.

Ataxic Cerebral Palsy

This is the least diagnosed type of Cerebral Palsy. Your child will have trouble tying his shoes, buttoning his shirt, cutting with scissors, and doing other tasks that require fine motor skills. He might walk with his feet farther apart than normal and have trouble with his balance and coordination. Your child may also suffer from “intention tremors,” a shaking that begins with a voluntary movement. For example, your child may reach for a toy, and then his hand and arm will start to shake. As he gets closer to the toy, the tremor worsens.

Hypotonic Cerebral Palsy

Unlike with other types of CP, you will notice that your baby has muscle control problems early in life. Her head seems floppy, and she will not be able to control it when sitting up. Her motor skills will be developmentally delayed.

It is suspected thatthis type of Cerebral Palsy is caused by brain damageor malformations that occur while a baby’s brain is still developing.

Mixed Cerebral Palsy

If your child does not “fit” into one of the above diagnoses, your doctor will consider him “mixed.” This is quite common.

Congenital Cerebral Palsy

If your child is diagnosed with congenital Cerebral Palsy, be aware that this is not a “type” of palsy, but rather it is a term meaning “birth defect.” In other words, your child’s doctor is saying that he developed Cerebral Palsy during development. It is not a condition that your child inherited from you or your husband or partner. And it is not caused by a medical error.

After once again reading those definitions, I would venture to say that many hydran children have a mix of all the various types throughout their lifetimes. Brayden has shown signs of Ataxic, Hypotonic, Athetoid Dyskinetic and it is definitely congenital. Every child with hydranencephaly varies in the degree of symptoms they have, just as any typically developing child varies, and that can change from one day to the next as well!

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