Dejerine-Sottas is a rare neurological disorder that is genetically inherited. It affects the nerves that connect the brain and spinal cord to muscles.
Nerves have a sheath covering them called myelin sheath which provides insulation. In this disorder the myelin sheath is destroyed thereby affecting nerve function.
The nerves enlarge and have an onion bulb like appearance. Genes that affect nerve development are mutated in this disorder.
The progression of the disease speeds up between 10-30 years of age. Since these nerves connected to muscles are affected, muscle weakness is one of the first symptoms followed by pain, numbness, tingling or burning sensation in extremities.
Loss of heat sensitivity, reduced muscle mass and absence of reflexes in legs are some of the other symptoms.
These symptoms spread to other limbs as well and individuals cannot walk or require support while walking. Occasionally there could be vision problems, hearing loss and delay in acquisition of motor skills in children.
Treatment for this condition is symptomatic. Surgery or foot bracing is provided to support joints while walking. Genetic counselling is advised for families with individuals suffering from Dejerine-Sottas. There is no known cure for this disease.
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What was the study about?
Here is a case report published in Paediatric Neurology, 2009 investigating the effect of oral curcumin in Dejerine-Sottas disease.
Burns et. al from University of Sydney and Institute For Neuromuscular Research were the first to report these findings in human case study.
The patient was a 15 year old girl with Dejerine-Sottas disease and she suffered from weakness, scoliosis (curvature of spine) and impaired respiratory function.
The safety trial lasted for 12 months. For first 4 months the girl received oral curcumin at a dose of 50mg/kg/day and 75mg/kg/day for the next 6 months.
Changes in muscle strength, disability of extremities, respiratory function, neurophysiological function and quality of life was observed.
What were the results?
No adverse effects were reported. Though little, but a positive improvement in parameters was observed.
Flexibility of knee and foot strength increased but hand and elbow strength decreased. Respiratory parameters and extremity disability was stable or reduced.
Parents reported that there was improvement in most domains and especially in self esteem in 12 months.
The patient also reported similar results and overall feelings of happiness and contentment were noted. Researchers felt that curcumin holds promise for such severe demylineating neuropathies.
How did this occur?
Curcumin has been identified as a neuroprotective agent- it protects from neurological diseases.
Khajavi et. al has investigated the role of curcumin in Dejerine-Sottas disease via animal model and cell culture studies. In their first study, they have investigated the toxic effect of mutations in the disease and how curcumin counteracts them.
Mutations in genes such as myelin protein zero (these genes are responsible for proper nerve development) lead to formation of abnormal or mutated proteins that accumulate in a structure found in cells called endoplasmic reticulum.
This accumulation leads to cell death of nerve cells and contributes to progression of the disease.
The scientists found that curcumin pretreatment of such cells that had accumulated mutated proteins, reduced the toxic effect of such mutations.
Curcumin prevented accumulation and supported release of such proteins from the cell structure. It also attenuated cell death.
Similarly in animal model, oral curcumin was found to partially mitigate the severity of the disease.
It reduced cell death and increased number and size of myelinated nerve cells (nerve cells having myelin sheath) in sciatic nerve (nerve that connects lower back to lower limb and is responsible for sciatica).
Also improvement in motor function and structure of nerve cells was observed. These findings show that curcumin could be a potential treatment in reducing symptoms of Dejerine-Sottas and related neuropathies.
Also in the study, improved quality of life and feelings of happiness, contentment and improvement in self esteem was reported. Curcumin works as anti-depressant and helps in favourably altering brain related chemicals to elevate mood and relieve anxiety and stress.
The problem of low bioavailability of curcumin could have been responsible for the limited improvement.
What does this mean?
Oral supplementation with curcumin can be therapeutic in Dejerine-Sottas disease and other motor function related neurological diseases. Including turmeric in diet can be of therapeutic importance in dietary approaches designed to ameliorate these conditions.
Read paper here: http://www.ncbi.nlm.nih.gov/pubmed/19748054