My Medical Condition

I was born with a malformation of the skull. This malformation is called Chiari’s Malformation. Arnold–Chiari malformation, or often simply known as Chiari malformation, consists of a downward displacement of the cerebella tonsils through the foramen magnum (the opening at the base of the skull), sometimes causing non-communicating hydrocephalus as a result of obstruction of cerebrospinal fluid(CSF) outflow. The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in the vasculature of the brain. It can cause headaches, fatigue, muscle weakness in the head and face, difficulty swallowing, dizziness, nausea, impaired coordination, and, in severe cases, paralysis. 

Chiari Malformation is a condition in which important parts of the back, bottom part of the brain extends downwards through the bottom of the skull where the brain joins the spinal cord. The brain structures that extend downwards in Chiari Malformation are the cerebellar tonsils, the pons, and the medulla oblongata. The cerebellar tonsils are two structures at the bottom part of the cerebellum. The cerebellum is an area in the back, bottom part of the brain that plays an important role in movement and coordination. The pons is very important for sleep and arousal and the medulla oblongata is extremely important for controlling breathing.

In Chiari Malformation the cerebellar  tonsils, pons and medulla oblongata stick out because there is not enough room at the back of the brain.  Normally the cerebellum, pons and medulla oblongata rest in an indented area of bone in the lower back part of the skull known as the posterior cranial fossa. However the posterior cranial fossa (skull)  is abnormally formed in people with Chiari’s malformation. With less space to be in, these areas are pushed downwards into the spinal cord.

Individuals with CM may complain of neck pain, balance problems, muscle weakness, numbness or other abnormal feelings in the arms or legs, dizziness, vision problems, difficulty swallowing, ringing or buzzing in the ears, hearing loss, vomiting, insomnia, depression, or headache made worse by coughing or straining and to some, they can have learning disabilities. Hand coordination and fine motor skills may be affected. Symptoms may change for some individuals, depending on the buildup of CSF and resulting pressure on the tissues and nerves. Persons with a Type I CM may not have symptoms. Adolescents and adults who have CM but no symptoms initially may, later in life, develop signs of the disorder. Infants may have symptoms from any type of CM and may have difficulty swallowing, irritability when being fed, excessive drooling, a weak cry, gagging or vomiting, arm weakness, a stiff neck, breathing problems, developmental delays, and sometimes learning disability.

The blockage of cerebrospinal fluid (CSF) flow may cause a syrinx (cyst) to form, eventually leading to Syringomyelia. Central cord symptoms such as hand weakness, dissociated sensory loss, and, in severe cases, paralysis may occur.

Having Chiari Malformation, like in my case caused me to have this insidious disease known as Syringomyelia. It is the cause of a life full of challenges for me.

Syringomyelia

Post-traumatic syringomyelia and tethered spinal cord can occur following spinal cord injury.  It can occur from two months to many decades after injury.  The results can be devastating; causing new levels of disability long after a person has had successful rehabilitation.  The clinical symptoms for syringomyelia and tethered spinal cord are the same and can include progressive deterioration of the spinal cord, paralysis or progressive loss of sensation or strength, profuse sweating, spasticity, pain and autonomic dysreflexia (AD).

Syringomyelia (sear-IN-go-my-EE-lia) a cyst or fluid-filled cavity forms within the cord.  This 

activity can expand over time, extending two or more spinal segments from the level of SCI.

Syringomyelia also occurs in people who have congenital abnormality of the brain called a Chiari malformation – during development of the fetus, the lower part of the cerebellum protrudes from the back of the head into the cervical portion of the spinal canal.  Symptoms usually include vomiting, muscle weakness in the head and face, difficulty swallowing, and varying degrees of mental impairment.  Paralysis of the arms and legs may also occur.

Surgery results in stabilization or modest improvement in symptoms for most people.  Delay in treatment may result in irreversible spinal cord injury.  Recurrence of syringomyelia after surgery may make additional operations necessary; these operations may not be completely successful over the long-term.  Up to one half of those treated for syringomyelia have symptoms return within five years.

Listed below are the complications that I suffer from because of having Chiari and Syringomyelia.

Occipital neuralgia, also known as C2neuralgia, or (rarely) Arnold's (fromArnold Chiari Malformation) neuralgia, is a medical condition characterized bychronic pain in the upper neck, back of the head and behind the eyes. These areas correspond to the locations of the lesser and greater occipital nerves. The greater occipital nerve has an artery that supplies blood that is wrapped around it - the occipital artery - that can contribute to the neuralgia. This condition is also sometimes characterized with diminished sensation in the affected area as well.Occipital neuralgia is caused by damage to these nerves. There are different ways in which they can be damaged including trauma. (In my case, the cause is from compression of nerves in the space between the vertebral bones of C1 and C2).

Autonomic Dysfunction

Your autonomic nervous system is made up of nerves that control those “automatic” things you need to do to survive. A few of those necessary things include blood pressure, heart rate, sweating, and digestion of your food. Autonomic dysfunction or dysautonomia refers to problems with this autonomic nervous system.

Myalgia- and Myositis- myalgia is muscle pain while myositis is the medical term for muscle inflammation. In myositis, inflammation damages the fibers of a muscle. This causes muscles to be weak by interfering with the ability of the muscles to contract. Although myositis can cause muscle aches and muscle tenderness, weakness is usually the dominant symptom.

In some cases, myositis is a short-term problem that goes away after a few days or weeks. In other cases, it is part of a chronic (long-term) condition. Chronic forms of myositis can lead to muscle atrophy (wasting and shrinking) and severe disability.

Radiculopathy, Radiculitis and Radicular Pain

Radiculopathy refers to a set of conditions in which one or more nerves is affected and does not work properly (a neuropathy). The emphasis is on thenerve root (radix = "root"). This can result in pain (radicular pain), weakness, numbness, or difficulty controlling specific muscles.

In a radiculopathy, the problem occurs at or near the root of the nerve, along the spine. However, the pain or other symptoms often radiate to the part of the body served by that nerve. For example, a nerve root impingement in the neck can produce pain and weakness in the forearm. Likewise, an impingement in the lower back or lumbar-sacral spine can be manifested with symptoms in the foot.(The worst part for me is around my brachial plexus).

Cervical spondylosis- also known as cervical osteoarthritis anddegenerative osteoarthritis, refers to the deterioration of the vertebrae and discs in the neck as we get older - specifically, the cervical spine, which is the section of spine in the neck. The edges of the vertebrae often develop small, rough areas of bone called osteophytes. As years go by the discs get thinner, increasing the risk of 
symptoms. The condition usually appears in men and women older than 40 and progresses with age. Men usually develop it at an earlier age than women do. It can lead to bouts of stiffness and neck pain.

Swollen neck joints can press or pinch nearby nerve roots or the spinal cord itself, resulting in pins and needles, and sometimes even pain in the limbs; in some cases there may be loss of feelings and coordination. Some patients may find walking difficult.

Cervical spondylotic myelopathy- is the most common cause of spinal cord dysfunction in older persons. The aging process results in degenerative changes in the cervical spine that, in advanced stages, can cause compression of the spinal cord. Symptoms often develop insidiously and are characterized by neck stiffness, arm pain, numbness in the hands, and weakness of the hands and legs.  

Cervical Spinal stenosis is an abnormal narrowing (stenosis) of the spinal canalthat may occur in any of the regions of the spine. This narrowing causes a restriction to the spinal canal, resulting in a neurological deficit. Symptoms include pain, numbness, paraesthesia (abnormal sensations) and loss of motor control. The location of the stenosis determines which area of the body is affected. Cervical spinal stenosis is more dangerous because it involves compression of the spinal cord.

Some of the common symptoms of stenosis are; Standing discomfort (94%)Numbness, Weakness, Bilateral symptoms, Discomfort above and below knee, Buttock / Thigh pain, Below the knee discomfort, "Shopping cart sign"- need to grab a shopping cart when going into a store in order to hold onto the cart and bend over relieving the pain in the legs. (I used to do that many years ago but later, my arms became weaker than my legs that I can’t hold on to anything. I had to give up my cane) While some of the neurological symptoms of stenosis are; numbness, lower limb numbness, weakness, diffuse or radicular leg pain associated with pares thesis, weakness and heaviness in buttocks radiating into lower extremities with walking or prolonged standing.

Central Cord Syndrome

Central cord syndrome (CCS), an acute cervical spinal cord injury (SCI), was initially described by Schneider and colleagues in 1954. It is marked by a disproportionately greater impairment of motor function in the upper extremities than in the lower ones, as well as by bladder dysfunction and a variable amount of sensory loss below the level of injury.

Although CCS has been reported to occur with particular frequency among older persons with cervical spondylosis who sustain hyperextension injury, it can be found in persons of any age and can be associated with various etiologies, injury mechanisms, and predisposing factors.

Central Pain Syndrome - a neurological disorder consisting of agonizing pain signals of many differing types at once:

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Burning

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Freezing

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Shocking

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Aching

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Crushing

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Spasticity

It has been characterized as the worst pain known to man. It is caused by damage to or injury of the Central Nervous System (CNS), which includes the brain, brainstem and spinal cord.

Peripheral Neuropathy- (this is the cause of my stomach getting big) and the reason for my edema. ( Edema or swelling- is the enlargement of organs, skin, or other body parts. It is caused by a buildup of fluid in the tissues. The extra fluid can lead to a rapid increase in weight over a short period of 
time as in days to weeks). Peripheral nerves carry information to and from the brain. They also carry signals to and from the spinal cord to the rest of the body. Peripheral neuropathy means these nerves don't work properly. Peripheral neuropathy may be damage to a single nerve. It may be damage to a nerve group.

Brachial neuritis – this is the cause of my pain around the root of my arms and chest area because of my cyst in the Thoracic region.

Thoracic or Lumbosacral neuritis and radiculitis- although my cyst only run down to the 7thThoracic vertebrae, from not being diagnosed early, my nerves were damaged down to the Lumbar region and Sacral region.

Degeneration of lumbar or lumbosacral intervertebral disc-these are additional conditions that I have to go through from having Syringomyelia.

Degeneration of cervical intervertebral disc

Chronic pain syndrome, abdominal pain, neck pain, leg pain, arm pain and back pain.

Despite having all of these symptoms and taking so many different kinds of medications, it is all but  invisible. Nothing shows on the outside that others always think we look healthy. Sometimes it's understandable, on the other hand it could get disappointing, even depressing when others ask us why we needed help. We just look normal, but in reality we're not.

Additional Information can be found at:

www.asap.org (American Syringomyelia & Chiari Alliance Project)

www. Christoprreeve.org (Christopher Reeve Spinal Cord Injury and Paralysis Foundation)

Spinal Cord Injury Primary Damage

www.chiari.org