Saturday, August 12, 2017
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
Apollo Hospitals, Hyderabad
Friday, June 16, 2017
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,
4. Speech impairment
5. Erosion, migration or fracture of the lead,
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
Sunday, May 4, 2014
CERVICAL AND LUMBAR DISC DISEASE
Disc is the soft tissue located in between the vertebral bodies.
A picture showing different types of disc problems.
Disc problems are common in cervical (neck) and lumbar (lower back) regions. Earlier, it was common in older people, due to more degeneration of discs in them. However, now-a-days, we see several younger people, even in their 20s and 30s, who come with disc problems. This is because of sedentary life style, lack of exercises, prolonged sitting (on account of job, computer use, etc), and sports injuries.
What are the common symptoms of disc diseases?
Disc prolapse or slipped disc can present with several symptoms:
In cervical disc disease, following symptoms are common:
1. Neck pain,
2. Pain in the arm, which may increase on coughing,
3. Tingling or pins and needle sensations in the arm,
4. Numbness in arm,
5. Weakness in the arm.
In lumbar disc disease, the following symptoms may occur:
1. Lower back pain,
2. Pain in the leg, which may radiate from lower back to the leg, also referred to as sciatica,
3. Tingling or numbness in leg,
4. Weakness of leg,
5. Leg pain, numbness or weakness may increase on walking (referred to as claudication), and get relieved on resting.
How is the diagnosis of disc disease confirmed?
1. Clinical history and examination by a neurologist are useful in suspecting the diagnosis.
2. MRI of spine (cervical or lumbar region, as the case may be) is the confirmatory test for disc disease.
An MRI scan of lumbar spine showing a prolapsed disc at L5-S1 level
What are the treatment options for disc disease?
Conservative (without surgery): It is useful to note that surgery is not needed in about 90% of cases.
Several measures are useful in relieving pain in patients with disc prolapse:
1. Rest- in severe cases, bed rest may be advised. In less severe cases, limited mobility within the house (for toilet and dining purposes) may be permitted.
2. Use of analgesics (such as aceclofenac, etoricoxib) and muscle relaxants (such as mobizox and myospaz forte) may help in relieving pain.
3. Specific medications that help reduce the nerve pain are pregabalin, gabapentin, duloxetine, etc.
4. Physiotherapy- measures such as IFT, ultrasound, traction, etc help reduce the pain in several people with disc prolapse.
In 10% of patients, medical treatment fails, then, surgery may be needed. Following are the specific indications for surgery in a patient with slipped disc:
1. Failure of medical treatment to adequately control the pain,
2. Progressive neurological deterioration, such as worsening of weakness or numbness, or bladder/bowel involvement,
Various types of surgeries are:
3. Spine stabilisation with instrumentation.
The choice of surgery depends on patient's symptoms, MRI findings and age.
General advice to patients with disc disease:
1. Avoid forward bending,
2. Avoid lifting heavy weights.
3. Lose weight, if overweight or obese
What is the prognosis of patients with disc disease?
In general, the outcome of patients with disc prolapse is good and most patients return to normal life after proper treatment. They are able to do their activities of daily living and are gainfully employed.
Surgery is also safe, however, I come across many people who have wrong notions about surgery. They have been mis-informed that after surgery, people get paralysed. In practice, this does not happen. However, if the case for surgery is not correctly chosen, there may not be good relief from pain after surgery also, a condition referred to as "failed back syndrome".
If you have any further queries, you may get back to me.
Dr Sudhir Kumar MD (Internal Medicine) DM (Neurology)
Senior Consultant Neurologist,
Apollo Hospitals, Hyderabad, India
Online consultation: http://bit.ly/Dr-Sudhir-kumar
Wednesday, January 29, 2014
Myasthenia gravis is an uncommon auto-immune disease, characterised by muscle weakness.
Common symptoms of Myasthenia Gravis
Myasthenia gravis presents with symptoms due to weakness of various muscles.
Common presentations of this include:
1. Drooping of one or both eyelids (usually, there is a difference in the degree of eyelid drooping). Drooping of eyelid is also called as ptosis.
2. Double vision, on looking up, down, left, right or sideways.
3. Slurring of speech or nasal twang in voice,
4. Difficulty in chewing food,
5. Difficulty in swallowing food, or nasal regurgitation of liquids,
6. Weakness of arms or legs,
7. Breathing difficulty,
8. Difficulty in holding neck straight (tends to droop forwards)
The sensations are normal in the affected parts of body.
Urinary and bowel control is preserved.
Please also note
1. Symptoms may vary at different times of day, most often they are worse in evenings,
2. There is fatiguability, symptoms get worse on repeating the same movements,
3. There may be periods (days to weeks) of remission, when all symptoms seem to disappear, only to return back later.
The following factors may trigger or worsen exacerbations:
- Bright sunlight
- Emotional stress
- Intercurrent illness (eg, viral infection)
- Medication (eg, aminoglycosides, ciprofloxacin, chloroquine, lithium, phenytoin, beta-blockers, statins)
How do we confirm the diagnosis of myasthenia gravis?
1. Clinical symptoms, as described above make us suspect a diagnosis of MG,
2. Tensilon test- where tensilon (edrophonium) injection is given, after which there is temporary improvement of symptoms such as ptosis,
3. Repetitive nerve stimulation- This test in done in the electrophysiology laboratory, where the muscle fatigue is shown (by seeing reduced amplitude with repeated stimulation of muscles),
4. Anti-Acetylcholine receptor antibody levels are elevated in about 80% of people. These antibodies are supposed to be released from thymus gland (located behind the upper chest wall in the centre). These antibodies prevent the action of acetylcholine on the muscles, making them weak.
5. CT scan of chest is done to look for enlargement or tumor in thymus gland.
6. Other less common antibodies seen in some patients with MG are anti-straited muscle antibody, anti-MuSK antibody, and anti-striational antibody.
What is the medical management of Myasthenia Gravis?
The medical management is classified into two categories:
1. Symptomatic treatment- to improve the symptoms. Pyridostigmine is commonly used.
We usually start a dose of 60 mg two times daily, and the maximum dose can be unto 720 mg per day.
Common side effects include increased salivation, abdominal pain and cramps, diarrhoea, muscle twitchings, etc.
2. Definitive treatment- is used to suppress the antibodies.
Steroids are commonly used.
Other agents used are azathioprine, mycophenolate, cyclosporine, methotrexate, and cyclophosphamide.
3. In patients with myasthenic crisis (severe disease requiring feeding tube due to swallowing problem), or requiring mechanical ventilation (due to respiratory distress), PLASMAPHERESIS (blood is purified, and antibodies are removed, something similar to dialysis) or IV IMMUNOGLOBULINS (injections are given by drip over five days) are required.
4. THYMECTOMY- the best and permanent treatment for MG is thymectomy operation. In this, the thymus gland located behind the sternum bone (chest wall) is removed by operation. This removes the source of antibodies and the disease gets cured in most people.
What is the outcome of myasthenia gravis after treatment?
Earlier, the disease had a high morbidity and mortality, as treatments were not available. However, now, with good treatments available, more than 95% people do well and are fully cured.
I hope this articles helps people with myasthenia gravis as well as their caregivers. I would be pleased to answer any further queries.
Please send email at email@example.com
If anyone prefers ONLINE CONSULTATION, please visit http://bit.ly/Dr-Sudhir-kumar
DR SUDHIR KUMAR MD (INTERNAL MEDICINE) DM (NEUROLOGY)
SENIOR CONSULTANT NEUROLOGIST
APOLLO HEALTH CITY
Phone (for appointment): 0091-40-23607777 (extension 6634/3011)
Online consultation: http://members.tripod.com/sudhirkumar_5/neurologist/id6.html