MOTOR NEURONE DISEASE (MND) is the name given to a group of related diseases affecting the motor neurones in the brain and spinal cord. Motor neurones are those nerve cells that control muscles, and their degeneration leads to weakness and wasting of muscles, causing increasing loss of mobility in the limbs, and difficulties with speech, swallowing and breathing.
Progress is relentless and generally rapid, with a life expectancy of between two and five years from the onset of symptoms. Approximately 20% of patients can survive for 5-10 years but the rate of progression varies greatly from one person to another. Death usually occurs due to respiratory failure.
The incidence of MND is 2 per 100,000 of total population, while prevalence is six per 100,000 of total population. Research has found that the incidence is higher in people aged over 50 years. Only 10% of cases are familial (inherited) with the remaining 90% sporadic. The male to female ratio is 2:1.
SYMPTOMS OF MOTOR NEURONE DISEASE
The symptoms of motor neurone disease usually follow a pattern that falls into three stages:
- the initial stage
- the advanced stage
- the end stage
There are different types of MND, each affecting people in different ways.
This is of course a diagnosis which needs a great deal of careful consideration given the poor prognosis. Nevertheless, it is equally important to ensure that the diagnosis is not unduly delayed. One study found that the average time from suspected diagnosis to confirmation was one year.
Diagnosis should be made after consideration of the clinical signs and symptoms together with investigations to exclude other causes.
Because of the very variable clinical presentation, the diagnostic criteria below have been devised, taking into account investigations to confirm the diagnosis and refute other possible causes (revised El Escorial Criteria):
- Evidence of LMN degeneration by clinical, electrophysiological or neuropathological examination.
- Evidence of UMN degeneration by clinical examination.
- Progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination
Together with the absence of:
- Electrophysiological and pathological evidence of other disease processes that might explain the signs of LMN and/or UMN degeneration.
- Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs.
There are no specific investigations that will confirm a diagnosis of motor neurone disease (MND). A range of investigations are carried out to confirm consistent features and exclude other possible pathologies, usually under the direction of a neurologist. It may take several months to decide that the clinical presentation, progression and investigation findings are consistent with the diagnosis. This cautious approach is understandable, given the prognosis of the illness and the devastation that being given the diagnosis may cause to a person’s life. The investigations below may be conducted during the course of making the diagnosis:
- Electrophysiological studies such as Electromyography (EMG) and nerve-conduction studies (NCS) will show a characteristic pattern but require careful interpretation, along with a consideration of the clinical features. In MND, EMG shows fibrillation and fasciculations. The motor units are polyphasic and have high amplitude and long duration. NCS should show normal motor and sensory conduction in MND. EMG is important in establishing the presence of widespread anterior horn cell damage that is unexplained by a single nerve, root or plexus lesion. This involves demonstrating evidence of acute denervation and reinnervation by examining at least two muscles in an affected limb, at least one muscle in a clinically unaffected limb and at least one muscle innervated by a cranial nerve, e.g. sternocleidomastoid or tongue. The assessment of thoracic paraspinal muscles by electromyography provides a useful strategy for differentiating MND from spondylosis because the thoracic paraspinal muscles are frequently affected in MND and spared in spondylotic amyotrophy. Electrophysiological findings should be given equal weight to the clinical findings in the diagnosis of the condition.
- CT and/or MRI scanning of the brain and spinal cord are useful in excluding other pathologies with similar presentations. Neuro-imaging is not yet considered to be sufficiently sensitive to assess responses to treatment but the European Federation of Neurological Societies recommends that it should be incorporated into the evaluation of research trials.
- Blood tests to exclude other conditions, such as vitamin B12 and folate levels, HIV serology, Lyme disease serology, creatine kinase assay, serum protein electrophoresis, anti-GM1 antibodies (multifocal motor neuropathy with conduction block), urinary hexosaminidase-A assay (Tay-Sachs) and a host of other more specialized tests for rare conditions.
- Muscle biopsy may be considered to exclude or to diagnose myopathic conditions.
Riluzole (a neuroprotective glutamate-release inhibitor) is the only drug of proven disease-modifying efficacy but its effects are modest, probably only prolonging lifespan by between 2 and 4 months. It may have a more significant effect on prolonging tracheostomy-free survival. It appears to be well-tolerated and of greater benefit the earlier it is started in the course of the disease. It has been shown to act by blocking muscle acetylcholine receptors.
Riluzole does not reverse the damage already done to motor neurons, and people taking it must be monitored for liver damage
Other medications may be used to treat symptoms of the disease; for example:
- Drooling may be reduced by the use of anticholinergics such as hyoscine.
- Muscle cramps and spasticity can be treated with agents such as diazepam, baclofen, tizanidine, phenytoin and quinine.
- Respiratory distress and the sensation of choking may respond to opioid medications but this must be balanced against their tendency to cause respiratory suppression; they are very useful to treat this symptom in the palliative phase.
- Depression associated with MND may respond to the use of antidepressant medications.
- Pain often goes under-recognised and undertreated. Mild to moderate pain can often be controlled using non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen. More severe pain can be treated using an opiate-based painkiller, such as morphine.
As motor neurone disease progresses, swallowing problems (dysphagia) will become so severe that you won’t be able to eat and drink normally. One widely used treatment for dysphagia is a feeding tube known as a percutaneous endoscopic gastrostomy (PEG) tube. A PEG tube is surgically implanted into your stomach through a small incision on the surface of the stomach. PEG tubes are designed for long-term use and last for up to six months before they need replacing.
In Ayurveda, health is defined as the state where physical body, senses, and psyche are in original or natural state with respect to body and function.
Although there is no precise equivalent for MND in Ayurveda, MND can be considered as ‘VATA’ predominant disease. Vata is considered as a chief factor for the physiological maintenance of the body. Vata biofactor is responsible for functions of the central, autonomic, and peripheral nervous systems. Vata controls the respiratory, blood, lymphatic, excretory, and reproductive systems, as well as all types of movements. It is also responsible for the cognitive and neocognitive function of the brain and secretion of various chemical neurotransmitters and hormones. This description of Vata resembles the functions of central, peripheral, and autonomic nervous systems.
The Vata diseases as described in Ayurvedic classics include a wide range of neurological morbidities, including inflammatory, degenerative, obstructive, and functional. Factors provoking Vata result in the instantaneous manifestation of diseases, which can even prove to be fatal.
Most of the signs & symptoms of MND like fasciculations, cramps, wasting, weakness, spasticity, bulbar symptom etc. match that of the classical signs & symptoms of Vata derangement described in Ayurveda. So the line of treatment in Ayurveda mainly focuses on the pacification Vata and bringing back the equilibrium between all the three Doshas (Vata, Pitta & Kapha).
‘CHARAKA-THE SPECIALITY AYURVEDA’ Institute of Panchakarma & Research, a traditional health care center, started treating MND/ALS cases since the year 2003. Our knowledge of MND has evolved greatly over the past few years. We have been continuously working on this disease and are committed for the better success in treating MND patients.
CHARAKA Ayurveda facility offers a very effective treatment methodology for MND, based on the classical texts of Ayurveda. Management of MND is usually aggressive and primarily consists of four procedures:
- samsodhan (cleansing through Panchakarma therapy)
- samsaman (palliative care through researched internal medicines)
- kaya kalp (rejuvenation)
- sattvavajaya (counseling/psychotherapy)
Along with these, strict diet & life style modification will be advocated.
Ayurveda therapy can be comfortably combined with Riluzole therapy as there is no interaction between Ayurveda medicines & Riluzole.
Today, CHARAKA stands to be the most experience Ayurveda facility in India for treating MND cases. Apart from treating, we are also educating and creating awareness among the MND patients and their relatives.
Inspite of the best possible treatment efforts, currently complete cure of MND in most of the cases is not possible as this disease as per Ayurveda comes under the criteria of ‘Kashta saadhya roga’ (difficult to cure).
***IMPORTANT ISSUE TO BE NOTED AND UNDERSTOOD BY ALL MND PATIENTS AND THEIR ATTENDENTS***
EARLY DIAGNOSED & TREATED CASES RESPOND TO THE MAXIMUM AND RATE OF SUCCESS WILL DECREASE WITH CHRONICITY OF THE DISEASE.