1. health and fitness
2. disorders

Hydranencephaly and Cerebral Palsy
by Alicia Harper
Hydranencephaly and Cerebral Palsy
One of the first bits of advice that I was given in the beginning of our
journey, and one of the many that stayed in my mind for many months
before I took heed to it, was that hydranencephaly would be easier to
explain by just saying Cerebral Palsy (CP). I never researched how much
truth was in this statement until the I tried for a year to explain what
hydranencephaly was to questioning individuals who turned out to not seem
to care as much as necessary for a full definition. I'd never actually known
how broad of a condition CP was until researching and discovering what an
umbrella of primary diagnoses this term actually blankets. It is a wee bit
frustrating, that so many rare conditions such as hydranencephaly exist and
are so easily classified into another group and forgotten about as an
individual condition...maybe for hydran that will change one day!
Cerebral Palsy is defined by the National Institute of Neurological Disorders
and Stroke (NINDS) as:
"...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 has a traumatic brain injury
(and many times has been shown to have had a stroke or other injury inutero)... I can definitely see how it would be easier to explain to curious onlookers. The main difference, and it's a big one, is that most children with
"simple" CP do not necessarily 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
which may or may not grow in to a more severe case, but others suffer more
severely with uncontrollable, and often painful muscle uncontrolled 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 smaller than normal,
or even total non-existence of the cerebellum. This type is directly correlated
to dysfunction of the cerebellum, with or without brain lesions, causing
various dysfunction dependent upon which portion of the brain is affected.
For example, in children who display balance and lack of control of eye
movement are said to have vestibulo-cerebellar dysfunction. While those
with a terrible gait have spino-cerebellar dysfunction, and those who are
unable to control voluntary movements are given the diagnosis of cerebrocerebellar dysfunction... all appropriately named by the area of the brain
affected, being displayed in the associated lack of muscle control.
There are also, I have found, many types of CP...to 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, 4MyChild: Help and Hope for Life (http://www.cerebralpalsy.org):
"Spastic Cerebral Palsy is the most common diagnosis. If your child’s CP is
“spastic,” her muscles are rigid and jerky, and she has difficulty getting
around. There are three types of spastic Cerebral Palsy:
Spastic diplegia — 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.”
Spastic hemiplegia — 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.
Athetoid Dyskinetic Cerebral Palsy is 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 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 which, 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 that this type of Cerebral
Palsy is caused by brain damage or malformations that occur while a baby’s
brain is still developing.
Mixed Cerebral Palsy is diagnosed 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 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!.