EDARAVONE- A NEW HOPE FOR PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS

EDARAVONE- A NEW HOPE FOR PATIENTS SUFFERING FROM AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Amyotrophic lateral sclerosis (ALS) is an uncommon degenerative disease of nervous system, mainly affecting the motor nerves. The common symptoms of ALS include weakness of arms and legs, difficulty in swallowing & speaking and breathing problem. The symptoms of ALS continue to get worse over time and most people die within 3-5 years after diagnosis, often due to respiratory failure.

                                          Stephen Hawkings (suffering from ALS)      AFP

There is no cure available for ALS. There is only one medication, RILUZOLE, which was approved for treating ALS in 1995. Riluzole can be used to slow down the deterioration in muscle strength. However, it has limited benefit in most patients. (In India, riluzole is offered free of cost to patients with ALS by Sun Pharmaceuticals). 

Now, there is a new hope for patients with ALS. A new medicine, EDARAVONE INJECTION, has been approved by US FDA to treat patients with ALS.

What is basis of Edaravone efficacy?

A research was conducted in Japan on more than 100 patients suffering from ALS. Edaravone injections were given for a period of six months. After six months, patients who received edaravone had better functional status and better quality of life (as compared to those who did not receive edaravone). 

Which patients with ALS would benefit from Edaravone injections?

Patients with ALS of less than two years duration, with mild disease severity, would benefit from edaravone. In addition, they should not suffer from any respiratory failure. 

What is the treatment regimen?

Patients are given edaravone injection 60 mg per day as intravenous infusion (given over 60 minutes) for 14 days. Then there is a gap of 14 days. Edaravone injection is supplied as 30 mg/20 ml vials (in India). In US, it is usually supplied as 30 mg in 100 ml. So, two vials would be needed per day. 

In second month, the injection is given on 10 out of 14 days. There is a gap of 14 days. This is continued for five months. 

So, in total, patient receives 64 doses of edaravone injections (60 mg each time) over a period of six months.

How long is the treatment continued?

As of now, there is efficacy and safety data for six months, so, it should be continued for total of six months, as per the schedule mentioned above. 

Is Edaravone treatment safe?

Yes, there are no serious adverse effects with edaravone. Minor side effects are similar to placebo. 

Do we need to reduce the dose of Edaravone in patients with kidney or liver disease?

There is no need to reduce the dose in patients with renal or liver function impairment. 

What is the cost of edaravone injections?

Each 30 mg vial costs about INR 400 (in India). So, the per day cost is about INR 800. The total cost of 64 days course of edaravone would be INR 51,200 (approximately 800 USD). Additional room rent, nursing charges, doctors fees, etc may be incurred. 

How about Riluzole?

Riluzole tablets should be continued together with edaravone injections. 

So, in summary, now we have a new drug for ALS, which is the first drug approved in 22 years for ALS after Riluzole. 

DR SUDHIR KUMAR MD DM

Consultant Neurologist

Apollo Hospitals, Hyderabad

drsudhirkumar@yahoo.com

www.facebook.com/bestneurologist/

04023607777/60601066

DEEP BRAIN STIMULATION (DBS) IN PARKINSON’S DISEASE

DEEP BRAIN STIMULATION (DBS) IN PARKINSON’S DISEASE

Major Symptoms of Parkinson’s disease

Parkinson’s disease (PD) is a progressive neurological disease, characterised by tremors, rigidity, slowness of movements and postural imbalance. Though it is more common after the age of 40, people younger than 40 can also get affected (when it is known as young onset PD).

Other symptoms of PD include low volume speech (sometimes totally incomprehensible), expressionless face, reduced blink rate, reduced arm swing while walking, stooped posture, small handwriting (called micrographia), short-shuffling steps, tendency to fall forwards. Many people also have pain and aches in the affected limbs. Sleep disturbance and restless leg syndrome are common comorbid illnesses in people with PD. Severe memory impairment is NOT a feature of PD, however, in advance PD, mild memory impairment may occur in about 10% of patients. Similarly, loss of bladder control or erectile dysfunction is not a feature of PD. When these features are present, one should suspect multiple system atrophy (MSA).

Diagnosis of PD

The diagnosis of PD is still best made on the basis of clinical examination by an experienced neurologist (preferably trained in movement disorders, though not a must). PET and DaT scans are available to help in the diagnosis of PD, but they are not superior to a good clinical examination by an experienced neurologist.

Treatment of PD

Treatment of PD is still primarily medical and DBS is not the first option.

The most effective medicine is levodopa-carbidopa combination. If a patient does not respond to levodopa treatment, we should doubt the diagnosis (it may not be PD). Even though levodopa is the most effective medication, we should delay starting it by 2-3 years, to avoid side effects and lack of efficacy later on. Other medications in use are pramipexole, ropinirole, trihexiphenydyl, selegiline, rasagiline, amantadine, entacapone, safinamide, etc.

Role of Deep Brain Stimulation (DBS) surgery in PD

DBS was approved for PD in 2002. In the past 15 years, about 1,35,000 patients worldwide have undergone DBS for PD.

What does DBS surgery involve?

A neurosurgeon places the leads (thin wires) that carry electrical signals to specific areas of the brain. Then, the surgeon places a battery-run neurostimulator (like a pacemaker) under the skin of the chest.

The surgeon may use a programming device to adjust the settings. You may have a device, similar to a remote control, which allows you to turn the system on and off and check the battery. You may also be able to adjust the stimulation within options programmed by your doctor.

A neurologist initially evaluates a patient to determine whether he or she is the right candidate for DBS surgery. Then, further evaluations include brain imaging (MRI, CT, PET, etc), neuropsychological testing, UPDRS scoring, etc. Once the patient is found to be suitable for DBS, he is referred to the neurosurgeon.

Which patients are likely to benefit from DBS surgery?

1.     The diagnosis of PD should be definite.

2.     Patient has had PD for five years or more.

3.     Patient continues to respond to levodopa, even though the response may or may not be good.

4.     There are motor fluctuations, such as on-off phenomena, with or without dyskinesia.

5.     Various medical treatments have not had desired benefit.

6.     PD symptoms are severe enough to interfere with activities of daily living.

Which patients are NOT likely to benefit from DBS surgery?

1.     Patients with atypical Parkinsonian symptoms,

2.     Patients with multiple system atrophy (where bladder and sexual dysfunction are prominent symptoms),

3.     Patients with progressive supranuclear palsy,

4.     Patients with dementia or severe cognitive impairment,

5.     Patients with unstable psychiatric illnesses,

6.     Patients with advanced PD, who are confined to bed/wheelchair; despite being on medications,

7.     Patients with NO response to levodopa therapy,

Who are the best candidates for DBS surgery in PD?

1.     Excellent response to levodopa therapy,

2.     Younger age,

3.     Mild or no cognitive impairment,

4.     Few or no axial (affecting neck or trunk) motor symptoms,

5.     Absence of or well controlled psychiatric disease.

Are there any complications of DBS surgery?

DBS surgery is generally safe if performed by a trained group of specialists. However, complications may occur in upto 3% of patients, which include:

1.     Bleeding (hemorrhage) in the brain,

2.     Infection,

3.     Stroke,

4.     Speech impairment

5.     Erosion, migration or fracture of the lead,

6.     Death

What to expect after DBS surgery?

Most patients report a reduction in severity of symptoms after surgery. Tremors, dyskinesia, slowness all respond to the surgery.

PD medications, however, can not be stopped even after DBS. Most patients still need to take medications, however, at much lower doses.

The benefits are seen at five years after surgery, however, the effect tends to wane in later years.

DBS does not alter the disease progerssion, and disease continues to get worse even after DBS.

Is MRI safe after DBS?

Yes, MRI can be safely done after DBS surgery.

What is the cost of DBS surgery ?

The cost of surgery is approximately INR 9,00,000 to 10,00,000.

Dr Sudhir Kumar MD (Med) DM (Neuro)

Senior Consultant Neurologist

Apollo hospitals, Hyderabad

http://www.facebook.com/bestneurologist/

drsudhirkumar@yahoo.com

04023607777/60601066

COMPARISON OF COST OF MEDICAL TREATMENT IN NEUROLOGICAL ILLNESSES (INDIA versus USA)

COMPARISON OF COST OF MEDICAL TREATMENT IN NEUROLOGICAL ILLNESSES (INDIA versus USA) (in US dollars; 1 USD=70 INR)

	INDIA	USA
Neurologist consultation fees	10-20	500-600
CT scan charges	30-60	1,000-1,600
MRI scan charges	150	2,600
EEG (routine)	30-60	500-600
Long term EEG recording	150	3,000
NCS/EMG	50-150	1,000-5,000
Botox (One vial cost)	300	525
Hospitalization for acute STROKE treatment	1,500-7,000	14,000-30,000

The costs in India are that of private hospitals and diagnostic centers. Also, the discussion pertains to Neurology treatments in urban areas and metros of India.

Costs are exorbitantly high in USA

It is obvious that the treatment costs are several times higher in USA, as compared to India. I agree that insurance companies cover most of the cost in USA, but individuals still need to pay at least 10-20% of the bills. You can imagine the fate of those who do not have medical insurance (about 12% of Americans do not have insurance). In 2007, 62% of filers for bankruptcy in US claimed high medical expenses. US spends about 10,000 dollars per year per person on healthcare. About 20% of GDP is spent on healthcare. The medical treatment costs in the US are the highest in the world. 

                                          (Source: Washington Post)

Waiting periods to see specialists/get procedures are long

In USA, there are long waiting periods to see a Neurologist, as well as, to get the procedures (EMG, EEG, Botox, MRI, etc) done. In India, one can see a neurologist, get the MRI done and get it reported by a Radiologist in a few hours! On the other hand, the average wait time to see a Neurologist in USA is 30-35 days. Getting the MRI done and its report would take several more days. The situation in UK and Canada are worse, where one may have to wait for 2-3 months to see a specialist and get an MRI done. 

The charges are higher in several other countries too, including Singapore, Australia and Thailand.

Indian Neurologists are well trained and experienced

Regarding the expertise of Neurologists, training in premier Indian Institutes such as AIIMS, PGI Chandigarh, NIMHANS, CMC Vellore, Sree Chitra, Tiruvannathpuram, JIPMER Puducherry, etc are at par with the best in USA. The clinical experience of treating neurological illnesses is several times more among Indian Neurologists, simply because they see more number of patients. There are about 2000 neurologists to cater to 1.25 billion people (one Neurologist for every 6,25,000 people). In comparison, US have about 18,000 neurologists for a population of 320 million (one Neurologist every 18,000 people). On an average, Indian neurologists see 3-4 times more number of patients as compared to American or British Neurologists.

In summary, Neurological treatment costs are much lower in India as compared to US and many other countries. The access to Neurology care is easy, and has little or no waiting times. Neurologists in India are well trained in the subject and have a vast clinical experience. 

DR SUDHIR KUMAR MD (Internal Medicine) DM (Neurology)

Senior Consultant neurologist

Apollo Hospitals, Jubilee Hills, Hyderabad

Phone: 0091-40-23607777/60601066
Online Consultation:  http://bit.ly/Dr-Sudhir-kumar
Email: drsudhirkumar@yahoo.com