Showing posts with label ataxia. Show all posts
Showing posts with label ataxia. Show all posts

15 February 2009

Stem cell transplant reverses early-stage multiple sclerosis

12 Feb 2009

Researchers from Northwestern University's Feinberg School of Medicine appear to have reversed the neurological dysfunction of early-stage multiple sclerosis patients by transplanting their own immune stem cells into their bodies and thereby "resetting" their immune systems.



"This is the first time we have turned the tide on this disease," said principal investigator Richard Burt, MD, chief of immunotherapy for autoimmune diseases at the Feinberg School. The clinical trial was performed at Northwestern Memorial Hospital where Burt holds the same title.

The patients in the small phase I/II trial continued to improve for up to 24 months after the stem cell transplant and then stabilised. They experienced improvements in areas in which they had been affected by multiple sclerosis including walking, ataxia, limb strength, vision and incontinence. The study will be published in the March issue of the Lancet Neurology.

Multiple sclerosis (MS) is an autoimmune disease in which the immune system attacks the central nervous system. In its early stages, the disease is characterised by intermittent neurological symptoms, called relapsing-remitting MS. During this time, the person will either fully or partially recover from the symptoms experienced during the attacks. Common symptoms are visual problems, fatigue, sensory changes, weakness or paralysis of limbs, tremors, lack of coordination, poor balance, bladder or bowel changes and psychological changes.

Within 10 to 15 years after onset of the disease, most patients with this relapsing-remitting MS progress to a later stage called secondary progressive multiple sclerosis. In this stage, they experience a steady worsening of irreversible neurological damage.

The 21 patients in the trial, ages 20 to 53, had relapsing-remitting multiple sclerosis that had not responded to at least six months of treatment with interferon beta. The patients had had MS for an average of five years. After an average follow-up of three years after transplantation, 17 patients (81 percent) improved by at least one point on a disability scale. The disease also stabilized in all patients.

In the procedure, Burt and colleagues treated patients with chemotherapy to destroy their immune system. They then injected the patients with their own immune stem cells, obtained from the patients' blood before the chemotherapy, to create a new immune system. The procedure is called autologous non-myeloablative haematopoietic stem-cell transplantion.

"We focus on destroying only the immune component of the bone marrow and then regenerate the immune component, which makes the procedure much safer and less toxic than traditional chemotherapy for cancer," Burt said. After the transplantation, the patient's new lymphocytes or immune cells are self-tolerant and do not attack the immune system.

"In MS the immune system is attacking your brain," Burt said. "After the procedure, it doesn't do that anymore."

In previous studies, Burt had transplanted immune stem cells into late-stage MS patients.

"It didn't help in the late stages, but when we treat them in the early stage, they get better and continue to get better," he said.

"What we did is promising and exciting, but we need to prove it in a randomised trial," Burt noted. He has launched a randomised national trial.

(Source: Northwestern University : Burt R. Stem cell therapy for patients with multiple sclerosis failing interferon A randomised study. Lancet Neurology. : February 2009)

18 February 2002

Cerebral Palsy

There are different types of Cerebral Palsy and they are:

Spastic Diplegia This type of cp primarily affects the legs.Hemiplegia This is a form of cerebral palsy that affects one arm and leg on the same side of the body

Double Hemiplegia This is used to describe people who have a weakness in all four limbs, with more involvement on one side of the body than the other.Quadriplegiathis is a form of cp that affects all limbs.

Athetoid This type of CP is characterised by tremors, unsteadiness, lack of coordination, and constant movement. People with Athetoid Cerebral Palsy often have speech difficulties as well.Ataxicthis type of CP is the least common form of cerebral palsy. Ataxia means having a lack of balance. People with ataxic CP have a disturbed sense of balance and depth perception. They usually have low muscle tone, a staggering walk and unsteady hands.I have Spastic Diplegia, which means I have tight muscles and this can make it difficult at times to move about. I take muscle relaxants daily to help reduce the high tone allowing me to move a little easier. I walk with the help of a frame and I use a wheelchair.

Spastic diplegia refers to a type of cerebral palsy that is a neuromuscular condition of hypertonia and spasticity in the muscles of the lower extremities, usually those of the legs, hips and pelvis. It results from brain damage at birth that prevents proper development of the pyramidal tract, meaning that certain nerve receptors in the spine are in turn unable to properly absorb the gamma amino butyric acid which would otherwise properly regulate tone in the affected areas.

Without GABA, affected nerves perpetually fire the message for their corresponding muscles to contract and tighten up. Doctor William John Little's first recorded encounter with cerebral palsy is reported to have been among paediatric patients who displayed signs of spastic diplegia. Above the hips, persons with spastic diplegia typically retain normal or near-normal muscle tone and range of motion, though some lesser spasticity may also affect the upper body, such as the trunk and arms, depending on the severity of the condition in the individual; additionally, because the leg tightness often leads to instability in ambulation, some extra muscle tension usually develops in the upper body, shoulders, and arms regardless of the fact that the upper body is not directly affected by the condition. Spasticity in the legs is rarely so great as to totally prevent ambulation; i.e., most people with spastic diplegia can walk. However, spastic diplegia does result in the signature "scissor gait" that some able-bodied people might tend to confuse with the effects of drunkenness.Spastic diplegia typically results from damage to the motor cortex or corticospinal tract sustained before, during, or shortly after birth. This damage is usually caused by asphyxia, hypoxia of the brain, premature birth, birth trauma, haematoma in the brain, or the presence of certain maternal infections during pregnancy. Genetic susceptibility may also play a part. Known post-birth causes of spastic diplegia may include exposure to toxins, traumatic brain injury, encephalitis, meningitis, other brain infections, and drowning or suffocation.

Major treatments for spastic diplegia include:baclofen (and its derivatives), a gamma amino butyric acid substitute injected into the spine or administered via an intrathecal pump; phenol, injected selectively into the over-firing nerves in the legs to reduce spasticity in their corresponding muscles; botox, injected directly into the spastic muscles; orthopaedic surgery to release the spastic muscles from their hypertonic state (these results are usually temporary because of the source of the spasticity being in the nerves, not in the muscles); Rhizotomy, a neurosurgery directly targeting and eliminating (cutting) the over-firing nerve rootlets and leaving the properly-firing ones intact. The term "spastic" describes the attribute of spasticity in spastic cerebral palsy, but has since been used extensively as a general insult to disabled people and/or as an insult to able-bodied people when they seem overly anxious or unskilled in sports (spazz). In 1952 a UK charity called The Spastics Society was formed; the charity changed its name to Scope in 1994.