IDIOPATHIC INTRACRANIAL HYPERTENSION

IDIOPATHIC INTRACRANIAL HYPERTENSION

What is Idiopathic intracranial hypertension (IIH)?

IIH is a condition characterized by raised intracranial pressure (ICP) of unknown etiology. It is also known as benign intracranial hypertension or pseudotumor cerebri.

What are the common symptoms of IIH?

The commonest symptom of IIH is headache. In some cases, visual blurring or double vision may occur.

What is the dreaded complication in patients with IIH?

If IIH is left untreated, there is a threat to vision. Partial or complete loss of vision may occur. In many of these cases, vision may not revert to normal even after treatment.

How is the diagnosis of IIH confirmed?

The diagnostic criteria for IIH, including those of the Dandy criteria as described by Dandy in 1937 and later modified, are as follows (source: Medscape)

·       There are symptoms and signs of increased intracranial pressure

·       There are no localizing neurologic signs (with the exception of a unilateral or bilateral sixth nerve paresis)

·       Cerebrospinal fluid (CSF) may show increased pressure, but there are no cytologic or chemical abnormalities

·       Neuroimaging reveals no structural cause or hydrocephalus

·       No other causes of increased intracranial pressure found through workup

Subsequent additions to these criteria include the following

·       The diagnostic lumbar puncture should be performed with the patient in the lateral decubitus position

·       Magnetic resonance venography should be included to rule out intracranial venous sinus thromboses

What are the common abnormalities of IIH on MRI/MR Venogram brain?

Brain MRI with gadolinium contrast venogram is the imaging modality of choice, as it can effectively rule out meningitis/meningeal infiltrates, hydrocephalus, mass lesions, cerebral venous sinus thrombosis, etc. In addition, there are certain abnormalities, which are typically seen in patients with IIH:

·      Partial empty sella

·      Flattening of posterior globe

·      Distension of perioptic subarachnoid space

·      Vertical tortuosity of orbital optic nerve

What is the role of lumbar puncture in the diagnosis of IIH?

Lumbar puncture should be done in left lateral decubitus. It confirms the elevated ICP, opening pressure >200 mm water. Lumbar puncture also helps in excluding meningitis.

What is the mainstay of treatment?

Medical treatment with diamox (acetazolamide) is the primary treatment to be started in patients with IIH. Starting dose should be 500-1000 mg per day and a maximum dose of 4 grams per day can be used. Common side effects include paresthesia and tingling.

Diamox to lower intracranial pressure is indicated when there is 

1) visual field loss on automated perimetry, 

2) transient visual obscurations, 

3) binocular diplopia, or 

4) pulsatile tinnitus.

Does weight loss help in patients with IIH?

Yes, a weight loss of 5-10% can lead to substantial reduction in reducing symptoms of IIH and papilledema.

Do patients with IIH need surgery?

There are a group of patients that need surgery. Patients with severe papilledema with impending threat to visual loss or those presenting with loss of vision require an early surgery. Another group of patients who may need surgery are those that continue to worsen despite being on adequate doses of acetazolamide.

What are the surgical options in IIH?

1.     Optic nerve sheath fenestration

2.     CSF diversion procedures such as theco-peritoneal or ventriculo-peritoneal shunt surgery.

Dr Sudhir Kumar MD DM

Consultant Neurologist

Apollo Hospitals, Hyderabad

drsudhirkumar@yahoo.com

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

MANAGEMENT OF MIGRAINE HEADACHES

Management of Migraine Headaches

There are two steps involved in ensuring the best outcomes for a patient with migraine:

1. Correct diagnosis, and 
2. Correct treatment.

Diagnosis of Migraine Headaches

Migraine is a common condition, affecting about 15% of women and 5% of men. Often, no tests are required for diagnosing migraine. Despite this, there is a significant delay in diagnosis of migraine in many cases. The correct diagnosis depends on the clinical features. Therefore, a good history, as given by the patient, is often sufficient for the diagnosis of migraine. The details can be read on my earlier post in May 2011

http://bestneurodoctor.blogspot.in/2011/05/how-to-diagnose-migraine.html

Treatment of Migraine Headaches

Starting appropriate treatment is equally important.

Many patients come to me telling that there is no treatment available for migraine or it can not be cured or I have to suffer with these headaches whole life and so on. So, they never take any treatment. Obviously, it is totally incorrect. There are excellent treatments available for treating migraine, and more than 95% of patients get better with proper treatment. You can read about these treatments later on in this post.

There is another group of patients who take only pain-killers, as and when they get headaches. This approach is not correct and may be harmful too, on account of three reasons:

1. Taking a pain-killer may help in reducing one episode of headache, however, it does not prevent the recurrence of headaches in future.
2. Pain-killers may cause side effects such as gastric ulcers, acidity, liver damage and kidney damage, if used for long.
3. Taking more than 15 tablets of pain-killers per month may actually worsen the headaches, a condition called as analgesic-abuse or analgesic-overuse headaches.

On account of the above, it is advised to restrict the use of pain-killers to as low as as possible.

Medical treatment of Migraine

1. If a person has only one or two episodes of headaches per month, then, there is no need of any preventive medications. Use of analgesics may be justified in these cases, as and when they get headaches. Common drugs in this category include-

• Paracetamol,
• Disprin,
• Zandu balm or tiger balm (very popular in India)
• Ibuprofen,
• Diclofenac,
• Vasograin,
• Rizatriptan,
• Sumatriptan

Any of the above can be used at the time of severe headaches. Some people also have vomiting, then ondansetron or domperidone tablets may be used.

2. Preventive therapy of migraine-

If a patient gets more than two episodes of headaches per month, then, it is important to start preventive medications (on daily basis) so that the headache frequency and severity can be minimised (or stopped).
Common drugs in this category include:

• Flunarizine
• Beta blockers such as propranolol,
• Topiramate,
• Divalproex sodium

In some cases, a combination of two medicines may be required.

Patients, who do not show adequate improvement with above, can be treated with botox injections. More details on this can be read in my previous post (October 2012)

http://bestneurodoctor.blogspot.in/2012/10/botox-treatment-for-migraine.html

I hope this article provides a little help to those with migraine. If you have any further queries, please mail me.

Dr Sudhir Kumar MD (Internal Medicine), DM (Neurology)
Senior Consultant Neurologist
Apollo Hospitals, Hyderabad
Phone: 0091-40-23607777/60601066
email: drsudhirkumar@yahoo.com