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Paralysis

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What is Hereditary Spastic Paraplegia?Hereditary spastic paraplegia (HSP), also called familial spastic paraparesis (FSP), refers to a group of inherited disorders that are characterized by progressive weakness and stiffness of the legs. Though the primary feature of HSP is severe, progressive, lower extremity spasticity, in more complicated forms it can be accompanied by other neurological symptoms. These include optic neuropathy, retinopathy (diseases of the retina), dementia, ataxia (lack of muscle control), icthyosis (a skin disorder resulting in dry, rough, scaly skin), mental retardation, peripheral neuropathy, and deafness. Diagnosis is primarily by neurological examination and testing to exclude other disorders. Specialized genetic testing and diagnosis are available at some medical centers.

Is there any treatment?

There are no specific treatments to prevent, slow, or reverse HSP. Symptomatic treatments used for other forms of chronic paraplegia are sometimes helpful. Regular physical therapy is important for improving muscle strength and preserving range of motion.

What is the prognosis?

The prognosis for individuals with HSP varies. Some cases are seriously disabling while others are less disabling and are compatible with a productive and full life. The majority of individuals with HSP have a normal life expectancy.

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What is Chiari Malformation?

Arnold-Chiari Malformation is a condition in which the cerebellum portion of the brain protrudes into the spinal canal. It may or may not be apparent at birth. Arnold-Chiari I type malformation usually causes symptoms in young adults and is often associated with syringomyelia, in which a tubular cavity develops within the spinal cord. Arnold-Chiari II type malformation is associated with myelomeningocele (a defect of the spine) and hydrocephalus (increased cerebrospinal fluid and pressure within the brain), which usually are apparent at birth. Myelomeningocele usually causes paralysis of the legs and, less commonly, the arms. If left untreated, hydrocephalus can cause mental impairment. Either type of Arnold-Chiari Malformation can cause symptoms of headache, vomiting, difficulty swallowing, and hoarseness.

Adults and adolescents who are unaware they have Arnold-Chiari I type malformation may develop headache that is predominantly located in the back of the head and is increased by coughing or straining. Symptoms of progressive brain impairment may include dizziness, an impaired ability to coordinate movement, double vision, and involuntary, rapid, downward eye movements.

Is there any treatment?

Infants and children with myelomeningocele may require surgery to repair protrusion of the meningeal sac into the spinal cord. Hydrocephalus may be treated with surgical implantation of a shunt to relieve increased pressure on the brain. Some adults with Arnold-Chiari Malformation may benefit from surgery in which the existing opening in the back of the skull is enlarged.

What is the prognosis?

Infants with very severe malformations may have life-threatening complications. Most patients who have surgery experience a reduction in their symptoms. Some patients may experience prolonged periods of relative stability.

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What is Alternating Hemiplegia? Alternating hemiplegia is a rare neurological disorder that develops in childhood, usually before the first 4 years. The disorder is characterized by recurrent but temporary episodes of paralysis on one side of the body. The paralysis can affect eye movements, limbs, or facial muscles. One form of the disorder, identified very recently, has a favorable outlook. It occurs primarily at night, when a child awakens, and is apparently related to migraine. These children have no other mental or neurological impairments. In more serious cases symptoms may include mental impairment, balance and gait difficulties, excessive sweating, and changes in body temperature. Seizures can occur. Sleep helps in the recovery from the periods of paralysis but the paralysis can recur upon waking. The cause of the disorder is unknown.

Is there any treatment?

Drug therapy including flunarizine may help to reduce the severity and duration of attacks of paralysis associated with the more serious form of alternating hemiplegia.

What is the prognosis?

Children with the benign form of alternating hemiplegia have a good prognosis. However, those who experience the more severe form have a poor prognosis because intellectual and mental capacity do not respond to drug therapy, and balance and gait problems continue. Over time, walking unassisted becomes difficult or impossible

What is Periodic Paralyses?

Familial periodic paralyses are a group of inherited neurological disorders caused by mutations in genes that regulate sodium and calcium channels in nerve cells. They are characterized by episodes in which the affected muscles become slack, weak, and unable to contract. Between attacks, the affected muscles usually work as normal.

The two most common types of periodic paralyses are:
Hypokalemic periodic paralysis is characterized by a fall in potassium levels in the blood. In individuals with this mutation attacks often begin in adolescence and are triggered by strenuous exercise or high carbohydrate meals. Weakness may be mild and limited to certain muscle groups, or more severe and affect the arms and legs. Attacks may last for a few hours or persist for several days. Some patients may develop chronic muscle weakness later in life.
Hyperkalemic periodic paralysis is characterized by a rise in potassium levels in the blood. Attacks often begin in infancy or early childhood and are precipitated by rest after exercise or by fasting. Attacks are usually shorter, more frequent, and less severe than the hypokalemic form. Muscle spasms are common.

Is there any treatment?

Treatment of the periodic paralyses focuses on preventing further attacks and relieving acute symptoms. Avoiding carbohydrate-rich meals and strenuous exercise, and taking acetazolamide daily may prevent hypokalemic attacks. Attacks can be managed by drinking a potassium chloride oral solution. Eating carbohydrate-rich, low-potassium foods, and avoiding strenuous exercise and fasting, can help prevent hyperkalemic attacks

What is the prognosis?

The prognosis for the familial periodic paralyses varies. Chronic attacks may result in progressive weakness that persists between attacks. Some cases respond well to treatment, which can prevent or reverse progressive muscle weakness

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What is Locked-In Syndrome?

Locked-in syndrome is a rare neurological disorder characterized by complete paralysis of voluntary muscles in all parts of the body except for those that control eye movement. It may result from traumatic brain injury, diseases of the circulatory system, diseases that destroy the myelin sheath surrounding nerve cells, or medication overdose. Individuals with locked-in syndrome are conscious and can think and reason, but are unable to speak or move. The disorder leaves individuals completely mute and paralyzed. Communication may be possible with blinking eye movements

Is there any treatment?

There is no cure for locked-in syndrome, nor is there a standard course of treatment. A therapy called functional neuromuscular stimulation, which uses electrodes to stimulate muscle reflexes, may help activate some paralyzed muscles. Several devices to help communication are available. Other treatment is symptomatic and supportive.

What is the prognosis?

The prognosis for those with locked-in syndrome is poor. The majority of individuals do not regain function.

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Paralysis