Conditions Explained

Arnold Chiari Malformation
Incomplete closure of the neural tube early in development can impact on the development of the entire nervous system. Arnold Chiari Malformation (also known as Chiari II Malformation) is an anomaly of the brain which occurs in almost all children born with myelomeningocele who have hydrocephalus. 

Arnold Chiari Malformation mainly involves the lower brain stem and lowermost portion of the cerebellum, but the anatomy of the whole brain is affected.

The brain stem is the origin of many of the nerves which control the heart, breathing, blood pressure and help control swallowing, sneezing and coughing.

The cerebellum controls the maintenance of posture and coordination of muscle action to produce precise coordinated movements.

When Arnold Chiari Malformation is present the brain stem is elongated and displaced into the opening of the base of the skull and top of the spinal canal. The brain stem, cranial nerves and lower portion of the cerebellum may be stretched or compressed and any of the functions controlled by these areas may be affected. The malformation also blocks the flow of cerebrospinal fluid causing hydrocephalus.

Complex Regional Pain Syndrome
Complex regional pain syndrome is believed to be the result of problems in the central or peripheral nervous systems. It can strike at any age and affects both men and women. Symptoms of the disorder include chronic pain, increased skin sensitivity, and changes in skin color or texture.

The brain produces cerebrospinal fluid (CFS) a clear, saltwater like liquid which flows in a circuit through the brain cavities (ventricles) and over the surface of the brain and spinal cord and is continuously reabsorbed by the body.

The fluid
  • protects and hydrates the brain
  • carries away waste from the brain cells
  • contains important chemicals and nutrients

Hydrocephalus occurs when there is an obstruction to the flow of CSF and fluid builds up within the cavities of the brain. this fluid build up causes pressure on the brain tissues and the bones of the skull.

Most children with myelomeningocele will have hydrocephalus. It is considered to be linked with Arnold Chiari Malformation.

Often the collection of fluid does not become severe until after the back has been closed so it may not become evident until the first few days or weeks after the child's birth.

Hydrocephalus is usually treated by insertion of a shunt. A shunt is a device that drains (via a one way valve) excess CSF from the brain to another part of the body (usually abdominal cavity).

Although a shunt generally works well, it may stop working if it disconnects, becomes blocked, or it is outgrown. If this happens CFS will begin to accumulate again.

Neuropathic Pain
Neuropathic pain is due to nerve damage, often severe and often described as burning

Spina Bifida
Spina Bifida is a birth defect that occurs because the normal development of the spine (growth from the right and left sides to join in the middle of the back) is incomplete, leaving a bony opening). The spinal cord, lying deep in the bone, may also be involved in this birth defect. The opening is often covered by skin and may not be visible to the naked eye (spina bifida occulta). 

With more significant abnormalities as may be seen in newborns, the opening is covered by some form of membranes over the spinal roots and may protrude through this opening. This is called myelomeningocele. Leg weakness, numbness and bladder and bowel control problems may be present. Meningoceles and myelomeningoceles  require surgical repair at infancy. They are generally associated with hydrocephalus, requiring shunting of the brain ventricles, the cavities containing spinal fluid that are normally present in the brain.

Syrinx refers to an abnormal fluid-filled space found within the spinal cord.

It generally occurs in the cervical region or thoracic region of the spinal cord. Syrinx's are commonly found in children with myelomeningocele. They are often asymptomatic however can cause various signs and symptoms.

Partial or Focal Seizures
These type of seizures start in one part of the brain (that is at a focal point of the brain) and affect that part of the body controlled by that part of the brain.

The seizure may involve the involuntary movement or stiffening of a limb, feelings of deja vu, or even an unpleasant smell or taste.

This glossary is intended for informational purposes only. I strongly encourage anyone with medical questions to seek professional medical advice.

Definitions from 
Chiari Malformation and Syringomyelia, A Handbook for Patients and Families Ulrich Batzdorf M.D.

SBH Queensland, PO BOX 8022 Woolloongabba QLD 4102

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