Wockhardt Hospitals Blog

Doctors at Wockhardt Hospitals led by Dr. K N Krishna, Senior Consultant in the Brain and Spine service successfully performed a rare and complicated spinal deformity correction surgery to save a 14 year old from leading a complete disabled life. Shah Rukh Khan a 9th standard student was suffering from Neurofibromatosis (an autosomal dominant disorder that affects the bone, the nervous system, soft tissue, and the skin) with a severe spinal deformity. There have been only a few cases reported the world over on surgical treatment for a rotational dislocation of the spine in neurofibromatosis.

About Neurofibromatosis:

While the cost of the surgery amounts to Rs 2.5 lakh, the hospital did not charge them much, except for consumable medicines. Shahrukh's family managed to borrow Rs 70,000 for his surgery and further treatment.

Movement is triggered through the pyramidal system, the basal ganglia and the cerebellum in the brain.These are discrete parts of the brain which perform specific but different functions, which are wired to each other for the performance of normal movement. The movement disorders are a diverse group of pathological dysfunctions in the nervous system, giving rise to any of these: an abnormal movement, a paucity of movement, an abnormality of muscle tone or a disturbance in posturalreflex.

The common movement disorders are Tremors, Chorea, Parkinson's disease, Dystonia and Writer's cramp.

The patient gives a detailed history of what has been occurring in terms of the symptoms. The investigation is followed by a neurological physical examination. Scales are used(basically written proformas) to assess the severity and type of movement disorder. Various movement sequences are recorded on video as a baseline before any treatment, to later compare the patient's performance in specific functions. This is followed by basic bloods tests, a CT/ MRI brain scan and EEG or EMG along with some specific tests that may be needed, depending on the diagnostic possibilities that the physician is thinking of.

1) Medications: Medications that either stimulate or inhibit the specific receptors for central nervous system chemicals (neurotransmitters) are the mainstay of treating such disorders

2) Botulinum toxin injection: This toxin is injected into muscles to partially paralyze them, and thereby control the abnormal movement.Several sites are injected in one sitting, and several such sessions may be required at 4 week intervals.

3) Stereotactic surgery: After detailed visualisation using CT/ MRI and mapping the brain, ablation (destruction) of certain parts of the brain nuclei and/ or its circuits will generally give relief from the abnormal movement.

4) Deep brain stimulation: The position of the basal ganglia nuclei are identified using CT/ MRI brain scans. Electrodes are placed onto specific brain nuclei and electrical signals are sent from a generator placed under the skin, in the chest. The patient has the option of controlling the current from the generator, for symptom relief.

The first task of the physician is to arrive at a diagnosis, or a highly probable diagnosis, using variouslines of investigation.The best line of treatment: Each patient would need to be tried on a specific combination of drugs which may vary from 1 to 3 months, to assess the best outcome with drug management.

Specific Medical Concerns on Movement Disorders

Following this minor adjustment in drug dosages may be required once every three months
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Likely outcomes: It is usually expected that the abnormal movement decreases to a remarkable extent. If the problem had been one of rigidity and paucity of movement, then movements should be more easily performed. A small sub-set of patients are not likely to improve on medications, for which deep brain stimulation or Stereotactic surgery should be considered to give optimal relief.

Movement disorders are generally progressive degenerative disorders, which means that the pathological course cannot be greatly altered, though significant improvement in function can be provided. However, the goal of management is to provide optimal relief and restore normal functionality, with the least side effects and thereby significantly improve the quality of life for the patients.

source:

The Department of Neurology at Wockhardt Hospitals

Dr. Chandran Gnanamuthu ,MD, DM, FIAN, FAAN DM PhD (Neuro Sciences)
Dr. C. Udaya Shankar,Specialist in Movement Disorder
Dr. Vijay Chockan,Consultant Neurologist Consultant Neurologist Consultant Neurophysiologist

Ankylosing Spondylitis Graphic Illustration

Ankylosing spondylitis (AS) was previously known as Bechterew's disease, Bechterew syndrome, Marie Strümpell disease

Ankylosing Spondylitis is approximately three times more common in men than women.It is generally seen in people in the age group of 20 to 40 years. However it is also known to affect children.

Ankylosing Spondylitis is believed to be genetically inherited. People with a particular gene called HLA-B27 are at a much higher risk of developing Ankylosing Spondylitis although not everyone with this gene will have the disease. There is no consensus in the medical community as to the other factors that causes the disease; some researchers put it down to a combination of factors i.e. genetics and environment.

The symptoms of Ankylosing Spondylitis are due to inflammation of the spine and joints . Hence pain in the lower / mid back, buttock and neck is usually the initial symptom. Pain is generally worse while resting and more prevalent in the early morning hours resulting in disturbed sleep. It reduces with physical activity and anti-inflammatory pain killers. The onset of pain is generally gradual, progressive with worsening over a period of time and in some cases the progression can be rapid and very disabling.

Patients with severe disease can develop complete fusion of the spine (known as Ankylosis), once fused the pain disappears but spinal mobility is lost resulting in severely compromised quality of life. The disease could affect hips and knees resulting in permanent damage.Some people with ankylosing spondylitis develop problems other than in the spine viz. eye inflammation (uveitis), skin rashes (psoriais) and chronic diarrhea (inflammatory bowel disease).

The diagnosis of Ankylosing Spondylitis is based on the patient's symptoms, a physical examination, x-ray findings and blood tests. The changes on X-ray are apparent only in the later stages of the disease. Magnetic resonance scan (MRI) is helpful to diagnose the disease in its early stages and also to delineate the severity.

Traditionally the treatment for Ankylosing Spondylitis involved the use of painkillers, physiotherapy and exercise. These only gave partial relief from pain but did not prevent the progression of the disease. Joint replacement surgery is recommended for those with severe hip or knee arthritis.

More recently, over the last few years, newer drugs (Biological therapy /anti-TNF therapy) have been discovered for this disease. The advent of biological therapy has revolutionized care for patients with this disabling disease. Infliximab (Remicade) and Etanercept (Enbrel) are the two drugs that are now available in India. They have been shown to cause dramatic reduction in pain, stiffness and resulting in greatly improved mobility of the spine. They are also believed to prevent the progression of the disease which had not been possible with the older treatments. This would hence reduce the intake of pain killers, reduce deformity and prevent the need for future joint replacement. In order to be more effective they should be used earlier in the disease rather than in the late stages.

It is important to diagnose and treat patients with Ankylosing Spondylitis early so as to enable the patient to lead a relatively normal life. The disease is chronic and hence long-term care is necessary.

More awareness needs to be created among the medical community and the general public to ensure early diagnosis and appropriate treatment.

To know more about Ankylosing Spondylitis and to schedule an appointment with Doctor Ramesh Jois,please write to enquiries@wockhardthospitals.net

picture courtesy : Albert Einstein Healthcare network