Forbesmedical.net: Neurology Information

What is a frontal lobe syndrome?
The frontal lobe is a part of your brain. It is found at the front of your brain, and takes up about 30% of the total brain volume. If the frontal lobe stops working properly, you can develope what is called a frontal lobe syndrome.

What does the frontal lobe do?
The frontal lobe has lots of different functions:

1. Personality
2. Emotional and Behavioural control
3. Planning abilities

If you want to work out a complex task such as how to decorate a room in your house you need to able to work out the size of the room, how much paint and brushes to buy, how to organise the room to allow you to paint it, and then actuially work out how to apply the paint to the walls. This sequence of events requires what is called strategic or planning abilities. The ability to work out this sort of sequence is a function of the frontal lobe

4. Control of eye movements
5. Speech
6. Motivation

What happens if my frontal lobe stops working?
If your frontal lobe stops working, you are developing a frontal lobe syndrome. You start to lose the functions listed above, so you could experience:

1. Change in personality

Some people become very excitable, and some become very withdrawn and quiet.

2. Loss of emotional control, and loss of social inhibitions

Some people may cry or laugh very easily, almost inappropriately. Others may become inappropriate with other people and make personal remarks about appearance that they would normally expect to keep to themselves. It is well known that people may get into trouble for these remarks before the frontal syndrome is diagnosed.

3. Apathy and loss of motivation
4. Slowness of speech
5. Difficulty with eye movement control

This can be difficult to diagnose, and symptoms of this are difficult to express. It causes particulr difficulty reacting to objects that rapidly come into view. Quite a lot of people will describe difficulty walking through crowds of people - they can’t avoid bumping into them, or may feel very uncomfortable driving. Driving is particularly hazardous as it is difficult to respond to sudden changes in speed or to cars which behave unexpectedly.

What causes a frontal lobe syndrome?
There are lots of causes of this. A brain scan (CT Scan) will rule out a brain tumour. A frontal lobe syndrome can follow a head injury. The neurodegenerations can cause frontal lobe syndromes e.g. Pick’s Disease also known as Frontal-temporal dementia, Progressive Supra-nuclear Palsy, ALS-type dementia, or small vessel cerebrovascular disease.
A frontal lobe syndrome can occur after a subarachnoid haemorrhage, especially if an anterior communicating artery aneurysm was the cause. There are rare cases of Multiple Sclerosis resulting in a frontal lobe syndrome.
Anti-potassium ion channel antibody syndrome can also cause a frontal syndrome. People with myotonic dystrophy can become apathetic and lack strategic planning function due to a frontal lobe syndrome.

Can a frontal lobe syndrome be treated?
This depends on the underlying cause. If the cause is trauma, stroke or a neurodegenerative disease, the answer is that treatment will not reverse the condition. The very rare cases of anti-potassium ion channel antibodies may respond to steroids. Drugs used in dementia, such as galantamine, memantine, rivastigamine or donezepil can be tried to imptrove thinking and motivation. I will use these on a trial basis i.e. no obvious improvement, prescription is stopped.

Apologies for the delay in posting this. It was posted on a previous version of forbesmedical.net on 6th February 2008, but when I changed web host, this post did not get transferred.

What is Brachial neuritis?
Brachial neuritis is a condition caused by inflammation of the nerves that travel from your neck to your arm. These nerves pass through a junction box of nerves called the brachial plexus. The brachial plexus is located on the side of your neck. Other names for brachial neuritis are Neuralgic Amyotrophy and Parsonage-Turner Syndrome.

What are the symptoms of brachial neuritis?
The classic features of brachial neuritis are a short period of very severe pain in the arm or shoulder. Often the pain is preceded by a viral type illness or vacciination a few days or weeks earlier. The pain is excruciating and symptoms can be mistaken for heart attacks, disc trouble in the neck, forzen shoulder or shingles. Once the pain settles (about 25% settle in one week, the rest within 4 weeks), there is then quite severe weakness of the muscles of the arm. Usually the weaknes isn’t noticed until the pain settles and you feel like moving the arm again. It is very common for people to hold the painful arm with their good arm until pain improves. This weakness persists for months and months. 80-90% of people have only one arm affected, 10-20% therefore have symptoms in both.

How is brachial neuritis diagnosed?
The diagnosis is based upon a typical story of a short period of intense pain followed by weakness. Unfortunately the condition is rare (about 10 cases per milion people per year), so unless the characteristic pain followed by thinning and weakness of muscles i noted, the diagnosis can be delayed for some time. A nerve conduction study or EMG test can provide evidence that the nerves of the brachial plexus (in the side of the neck) have been damaged. Sometimes an MRI of the neck is performed to make sure that there isn’t pressure on nerves in the spins from a disc ro “wear+tear” (spondolysis - in spondylosis the pain is usually more persistent and doesn’t resolve as quickly as brachial neuritis).

Can brachial neuritis be treated?
If there is a long delay between the first symptoms and a diagnosis, no medicines are offered as the inflammation will have settled and the nerves will have to make their own slow recovery. If the condition is diagnosed early, then a short course of steroids or a drip containing immunoglobulins (this a an immune system treatment obtained from blood donors) will be used.
An assessment from a physiotherapy can be very helpful to make the best of the remaining function of the arm. Occupational therapists can also help with tactics and devices to allow patients to dres or feed themselves when one or both arms are weak.

What is the long term outlook (prognosis)?
Most people (about 75%), if left long enough (usually 18 to 24 months) will make a fairly good recovery. Recovery is almost always slow. Persistent pain in the arm is the exception, rather than the rule. It is known that recovery may take several years in the worst affected cases. Persistent pain can occur,, but is usually related to the shoulder joint coming out of position due to the muscle weakness.

Patient advice pages - I couldn’t locate any that said anthing more than I’ve said here! If you can attempt to read the medical articles you may get more detail here.

• van Alfen’s series of cases published in the journal called Brain 2006;129:438-450 (free full text)
• Recent review article from Practical Neurology only a summary can be read, full text requires subscription.
• Neuromuscular Website University of St Louis, USA

Here’s a list of medicines that people have tried when treating Hypnic Headache.

Obviously the first-line drug is Lithium, and doses of 200mg to 400mg Lithium Carbonate (I’ve used Priadel TM) are usually sufficient.

PLEASE NOTE - these are unlicensed uses for these medications. They must only be used following consultation with your doctor, and I cannot be held responsible for any harm suffered as a result of using any of these medicines. I am only making observations - I am not recommending them!

Here’s other regimens gleaned from recent publications in medical literature:

1. Prednisolone 25mg daily for 2-4/52, then 12.5mg for 2 weeks
2. Lorazepam 2.5mg
3. Caffeine 40-60mg
4. Atenolol 25mg
5. Phenobarbital 40mg
6. Aspirin 325mg + cup of coffee!
7. Sumatriptan 50mg
8. Indomethacin 50mg at night
9. Gabapentin 300mg
10. Pregabalin 150mg
11. Rofecoxib 25mg

I’ve been asked about the rules governing use of medicines and the World Anti-Doping Agency (pdf file, Adobe reader is required) .
Of all the medicines I ever prescribe, these are the onesmost likely to fall foul of the rules and the diseases I would treat:

1. beta-blockers
• Migraine
• Essential tremor
2. Opioids
• Buprenorphine (Butrans patch)
• Fentanyl (transdermal patch)
• Chronic neuropathic pain
• Corticotrophins
• Prednisolone
• Methylprednisolone
• Multiple Sclerosis
• Bells Palsy
• Vestibular neuronitis
• Myelitis
• Optic neuritis
• Chronic inflammatory neuropathy
• Cluster headache
• Diuretics
• Acetazolamide
• Idiopathic Intracranial Hypertension
• Epilepsy
• Stimulants
• Modafanil
• Narcolepsy
• Idiopathic hypersomnia
• Fatigue associated with multiple sclerosis
• Fatigue associated with Parkinsons Disease
• Selegeline
• Used in early Parkinsons Disease (I don’t routinely prescribe this!)
• Cannabinoids
• Nabilone
• Multiple Sclerosis

As far as I can see these are the ones most likely to fall foul of their rules. This is only meant as a guide. If you are uncertain, you should seek advice from the WADA or a professional sports and doping advisor.

The latest news on anti-doping rules will appear here - updated using newsfeed software:
Anti-doping body warned about Jamaicans - The Age

RTE.ie
Anti-doping body warned about Jamaicans
The Age, Australia - 16 hours ago
VICTOR Conte, the jailed mastermind of the Balco drugs scandal in the US, says he warned the World Anti-Doping Agency about a drugs supplier working with ...
We are winni...

New head of USA Track and Field is trying to clean house - Kansas City Star

New head of USA Track and Field is trying to clean house
Kansas City Star, MO - 3 hours ago
For example, Jamaica authorized an official anti-doping agency exactly nine days ago. I'm not kidding - on the third day of the 2008 Games. ...

Olympics drug testing 'unable to detect' cheating athletes - Bangkok Post

Xinhua
Olympics drug testing 'unable to detect' cheating athletes
Bangkok Post, Thailand - 19 hours ago
The Caribbean country did not have an independent anti-doping agency until a week ago. We may still have to wait a long time to really find out wh...


Newsfeed display by CaRP

This is a quick overview of Parkinson Plus Disorders.

What do I mean by a Parkinson Plus Disorder?
The phrase Parkinson Plus disorder is used to describe people who develop symptoms that are similar to ordinary Parkinson’s Disease, but they are actually suffering from a different condition. Patients with Parkinson Plus Disorders may report tremor, falls, stiffness, poor balance, slowing up of movements, or confusion can be observed. Other patients (who turn out to have multisystem atrophy) may have problems with bladder or sexual function. In the early stages of Parkinson Plus, your doctor (GP or neurologist) may actually have said that Parkinson’s Disease is suspected, but over time it becomes apparent that the problem is a more complex.

What are the names of the Parkinson Plus Disorders?
There are 4 main conditions that can mimic Parkinson’s Disease - Small vessel cerebrovascular disease, Progressive Supranuclear Palsy, Multisystem atrophy and Corticobasalganglionic degeneration. Of these four, 3 are commonly included in the term Parkinson Plus Disorder. These three conditions are:

1. Progressive Supranuclear Palsy
2. Multisystem Atrophy - type P
3. Cortico-basal-ganglionic degeneration

Can Parkinson Plus Disorders be treated?
Unfortunately these conditions are not regarded as treatable. In about 20% of cases there may be some improvement in symptoms with medicines normally used in ordinary parkinson’s Disease (like Madopar or Sinemet).

How are Parkinson’s Plus Disorders Diagnosed?
The diagnosis is made after listening to a patient’s history and performing a neurological examination. A brain scan may not show any obvious abnormality in the early stages of these conditions. It is not usually possible to give a definite diagnosis of a Parkinson Plus disorder until after two visits or more to out-patients - I need to have time to see if there has been a change in the patient’s condition.

Where can I get more information?
These listed sites are UK-based and have helpful contacts listed.

1. Progressive Supranuclear Palsy (PSP Europe)
2. Multisystem Atrophy (Sarah Matheson Trust)

www.wemove.org is a US based charity. They have comprehensive information. It includes info on cortico-basal-ganglionic degeneration. However, it is more detailed, and I know that some of my own patients have found them to be a bit over-stated - this just reflects the difference in culture between Northern Ireland and the USA - what they say is factually correct.

This is for people who have been told they have a Chiari (also called Arnold-Chiari) malformation

What is Chiari Malformation?

A Chiari malformation is where the lowest part of your brain - called the “cerebellar tonsil” - has developed in such a way that it sits in the hole at the base of the skull called the “foramen magnum”. The surgeon who first described it was called Dr Chiari. There are four types of Chiari, but the most common one is Type I, and this post is about type I malformation.

What is the significance of a Chiari Malformation?
Nowadays the most common way a Chiari malformation is diagnosed is when it is detected during an MRI scan - usually performed for some other reason. Most Chiari malformations do not cause symptoms. Since the widespread use of MRI in the last 10-15 years, a Chiari malformation can be seen in just under 1% of people having MRI scans.

What symptoms can a Chiari Malformation cause?
In most people, there are no symptoms. Symptoms can occur if the flow of brain fluid (called cerebrospinal fluid) is affected. The classic symptom is called “cough headache”. This is where the sudden increase in pressure inside the skull, caued by a cough, is not allowed to settle quickly (due to the blockage caused by the Chiari malformation) and there is a sudden brief pain in the head provoked by coughing.
Other symptoms attributed to Chiari malformation include: pain in the back of the head and problems with balance. However a lot of symptoms have been listed in association with Chiari malformation. As the Chiari Malformation is a fairly common finding, it is not certain, to me, how many symptoms can reliably be attributed to an abnormally low sited cerebellar tonsil. I am more confident attributing symptoms in those cases where the cerebellar tonsils are quite tightly packed into the foramen magnum. I am very reluctant to attribute symptoms in people with very small amounts of cerebellar tonsil descent.

Can a Chiari Malformation be treated?
Most people do not need treatment. However, if you have a “tight” foramen magnum and classic symptoms, surgery might be offered. Usually this surgery invloves removing some of the bone to widen out the foramen magnum (the large hole in the bottom of the skull through which your spinal cord passes). This surgery is usually performed by a neurosurgeon - a separate referral is made to consult with a neurosurgeon.

Where can Ir ead more about Chiair Malformation?

Try these links for more information:

• NeurosurgeryToday.org Chiari information
• Mayo Clinic Chiari Information
• Chiari People blog
• Conquerchiari.org

These sites also contain a lot of information about syringomyelia, which many people with Chiari also have. However, I have restricted this post to isolated Chiari type I malformation.

Patient Information from the UK
There is a UK site which is devoted to Syringomyelia and Chiari

• The Ann Conroy Trust

Their information is very good, but says a lot more about the condition called syringomyelia than Chiari Malformation. However, there is a good testimony from a lady called Sally diagnosed with type I Chiari who eventually decided to undergo foramen magnum decompression . The actress Joanna David also is a patron of the Ann Conroy trust and she also had foramen magnum decompression.

Latest News on Chiari Malformation
I’ve set up this “news feed” to alert you to news items on people with Chiari malformations. NB - the feed uses a filter, so may include items about people with the surname “Chiari” - sorry for that. This news feed is updated automatically each time news on Chiair appears on web news sources.

Area highlights - Reading Eagle

Area highlights
Reading Eagle, PA - 1 hour ago
The Chiari People of PA will host Walk to Find a Cure for Chiari at the Fairgrounds Square Mall on Sept. 21. Chiari is a painful brain disease that causes ...

On TV early, Bogdanoff says it isn't a sign she's in trouble in ... - Sun-Sen...

On TV early, Bogdanoff says it isn't a sign she's in trouble in ...
Sun-Sentinel.com, FL - Aug 18, 2008
Chiari has taken in $49368, plus $23000 he lent to his campaign. He's spent $47332. Bogdanoff said her advertising shows more that she's "done w...

Q&A with Lisa Vassallo: Successfully lobbied the governor to ... - Norwich Bu...

Q&A with Lisa Vassallo: Successfully lobbied the governor to ...
Norwich Bulletin, CT - Aug 12, 2008
M. Jodi Rell making August Arnold-Chiari Malformation Month. She was diagnosed with the condition last year. By GREG SMITH Lisa Vassallo of Ledyard ...


Newsfeed display by CaRP

What is nummular headache?
This is very rare, but like most headache complaints not serious। The word “nummular” means “coin-shaped” and is an accurate description of the area of pain. It is centred on a small area of the surface of the head. If touched the area can feel hypersensitive, and in some cases the hair may thin a bit. The pain can be burning, shooting, stabbing or dull.

Why does it happen?
Inflammation (irritation) of a small nerve in the scalp is thought to be the cause. The main thing is that it is not caused by serious disease.

Can it be treated?
Usually this pain will go away on its own. If the pain levels are high, or if the pain persist for a long time medicines can be prescribed. Indomethacin or other anti-inflammatory medicines (Ibuprofen, Diclofenac) are quite likely to help. Lyrica or Gabapentin can also be used if the pain burns or the scalp remains hypersensitive after a course of anti-inflammatory medicine. Local injection of botulinum toxin (the same stuff for flattening wrinkles!) have made this pain better in people who did not respond to the medicines mentioned earlier.

Is it serious - will I need a brain scan?
Brain scans are not needed. This unusual pain is not due to a brain tumour.

Do I need to see a neurologist regularly?
Like other rare headache disorders, a neurologist will usually confirm the diagnosis and give your general practitioner advice on treatment options. Regular attendance at the neurology hospital clinic is not necessary. Sometimes people with nummular headache will also experience migraine or tension-type headache, and these can treated in the usual way, with advice from your family doctor if necessary.

Dr Pareja’s 2007 article on nummular headache

What is Parkinsons Disease?
Parkinsons Disease is a disease of the brain. It causes nerves which control movement to stop working, and results in tremor, stiffness and reduced mobilty or poor balance.

What causes Parkinson’s Disease?
The exact cause is not completely understood. However, it is known that cells in the brain which contain a chemical called dopamine stop working due to a build up of a protein called alpha-synuclein. The main group of nerves to stop working are called the nigro-striatal pathway. A lot of research is ongoing to look at how alpha-synuclein builds-up.

How is Parkinson’s Disease diagnosed?
The diagnose is usually suspected if you have at least one fo the following symptoms:

1. Tremor (a shake of your hand, and it is common for only one hand to be affected before the other)
2. Stiffness of slowness of movement: people will complain they are nt as sharp or quick as they used to be and may find fine movements difficult like doing up buttons or lifting coins from purse or pocket. Hand-writing may become scribbly or very small.
3. Poor mobility or falling easily. Walking can become slow and awkward and you might feel like you could easily fall, or may have fallen on one or more occasions.

If you have these symptoms your family doctor or hospital doctor will perfroma neurological examination to look for signs of Parkinson’s Disease.

If you have a story and examination consistent with Parkinson’s Disease, your doctor may be able to tell you this straight away without doing any tests.

Tests of brain structure (CT Scan) or brain function (DaTSCAN) can be helpful to rule out other causes of tremor, slowness or poor balalnce. These scans are not always needed if you examination is very typical for Parkinsons Disease.

Can Parkinsons Disease be treated?
The underlying loss of nerve cells due to alpha-synuclein protein build up is not treatable at present. However, the symptoms of tremor, stiffness and slowness can be treated by medicines. There are 4 main types of medicine used to treat Parkinson’s Disease. The idea is that they try to make your brain think that there is dopamine back in your system. This is acheived in 3 different ways:

1. Drugs that trick your brain into thinking there is dopamine present - called dopamin agonists - Pramipexole, Ropinorole, Rotigazone, Pergolide, Bromocriptine, Cabergoline
2. Drugs that replace the brains dopamine using a chemical called Levo-Dopa, also known as SInemet, Madopar, Co-careldopa, Cocarbidopa
3. Drugs that make any dopamine that is left work harder by stopping breakdown of dopamine. COMT-inhibitors (entacapone, tolcapone) and MAOI inhibitors (selegeline, rasagaline)

The best one for you will depend on your other medical history, age and what other medicines you are on or have previously tried. I normally start with pramipexole or Sinemet.

More advanced treatments include apomorphine subcutaneous pumps (about 60 cases in Northern Ireland with these pumps), and in exceptional cases (about one or two per year from Northern Ireland (population 1.7million) brain surgery with deep brain stimulation.

Stem cell treatment is being researched but I think this is still a long way off.

Where can I get more information?
If you are looking for more information about Parkinson’s Disease, I’d recommend the : Parkinson’s Disease Society website which is a UK based charity.
The 3 largest USA based sources of information on Parkinson’s are at

1. WeMove.org
2. National Parkinson Foundation
3. The Michael J Fox Foundation

.

All have excellent resources, including videos and easy to read patient information. Wemove.org also describes many other conditions where control of body movements are affected like dystonia, tremor or ataxia. Specialised information for medical professionals can also be found at the Movement Disorders Society home pages, although subscriptions are usually required to access the higher quality materials.

As far as possible, people with epilepsy should be aiming to live as normal a life as possible and this includes holidays!
Epilepsy Action have written a nice summary on travelling abroad, which I recommend. On that page they have suggested 4 travel insurance companies, which I’ve also added here (at the bottom of this paragraph).
The biggest problem is that ordinary travel insurance will exclude pre-existing medical conditions, including epilepsy. You will need to pay extra to be covered for travelling with epilepsy.
I’ve made a list of companies that might be more helpful. Epilepsy Action do have a Travel Insurer (Tyser) they recommend, but last time I looked this was not available (Thursday 5th June 2008).
Other things to remember before you travel:
1) Make sure you are travelling with someone who knows you have epilepsy
2) Make sure that the people with you know what to do in the event of you taking an epileptic seizure
3) Take enough medicines with you in your hand-luggage so that if your suitcase is lost you have enough to keep you going for a week (or long enough until you can get a supply from a chemist)
4) Avoid drinking too much alcohol - epileptic seizures can be brought on after heavy drinking
5) Ensure your travel insurance cover includes treatment for an epileptic seizure and the consequences of an epileptic seizure.

Epilepsy Action Suggested Travel Insurers:
Essential Travel - tel: 0870 343 0024
Medicover Travel - tel: 0870 735 3600
Free Spirit - tel: 0845 230 5000
Medici - tel: 0870 890 2991

I’ve added more who advertise as covering pre-existing medical conditions including epilepsy - let me know who was helpful and we could give them a “star” rating!
I tested one company for a hypothetical case with active epilepsy (more than 10 seizures in the last month, 1 hospital admission last year, epilepsy for more than 12 months and 3 medicines - the quote was £1200 for a 2 week trip. I then found Free-Spirit after reading about this families problem with obtaining epilepsy cover for their daughter. The quote came out at £198 for an adult (with no health problems) and a child with severe epilepsy (more than 10 seizures in last month, one hospital admission, losing consciousness during a seizure, on one medication).

Here’s other insurers who mention pre-existing medical conditions:

• allcleartravel.co.uk
• Tyser’s but Epilepsy Action said on 5th June 2008 the scheme was not available at present.
• MoneySupermarket have this list of insurers for people with pre-existing medical conditions, including Free-Spirit.com

.

Please let me know if you have any success with this list. I am not in any way endorsing any insurer, but like everyone else we are trying to find a helpful and fair insurance for you.
RF

This is for people who have been diagnosed with trigeminal sensory neuropathy. This should not be confused with the very painful condition called trigeminal neuralgia.

What is Trigeminal Sensory Neuropathy?
Trigeminal Sensory neuropathy (TSN) is an inflammation of the nerve to your face, which causes symptoms of persisting numbness affecting one side of the face. It usually comes on over a few days or weeks, often without causing any real problem. People usually ask for advice when they realise the the nunbenss is not going away. By the time to see me in neurology clinic they usually have had symptoms for several months.

Why is it called Trigeminal Sensory Neuropathy?
The main nerve which provides sensation to your face is called the trigeminal nerve (tri = three, the nerve has three main divisions). Sensory means that it only causes numbeness or tingling and not weakness. Trigeminal sensory neuropathy is a disorder of the sensation experienceed through the trigeminal nerve!

What causes Trigeminal sensory neuropathy?
In most cases no cause is found. It is possible that the trigeminal nerve could be infected with a virus, and the virus causes some sort of change within the nerve which leads to the symptoms. However, this is not proven. Inflammation of the nerve could start on its own. At present this is thought to be the most likely cause.

Are there any conditions associated with Trigeminal sensory neuropathy?
Yes. When the condition was first described, it was often associated with some joint or rheumatic disease like scleroderma or lupus. In modern practice these conditions are not commonly found. In my own practice over 90% of cases do not have an underlying rheumatic disease.

Can trigeminal Sensory Neuropathy be treated?
Most cases are not treated as the side-efects of medicines probably out-weigh any benefit. Most people are looking for an explanation rather than treatment. If the sensation has a painful or irritating quality to it, medicines to reduce the unpleasant sensation can be used. I usually would try Gabapentin, or Pregabalin or a low dose of amitryptiline. The aim of these tablets is to take the edge off the sensation, they will not remove the sensation completely.

Do I need any tests to diagnose Trigeminal Sensory Neuropathy?
The diagnosis is made from listening to your story and examinaing your face and nerves. To rule out another cause of the symptoms a CT Brain scan or MRI Brain scan may be performed. It is rare to find an underlying problem. Sometimes blood tests to look for an associated t=rheumatic condition are performed, but often your Family Doctor (GP) will have done these before you get to the neurology clinic.

Where can I get more information?
There are not many sites that discuss trigeminal sensory neuropathy. One of the more recent papers was written in 1987!
If anyone knows of any groups that discuss trigeminal sensory neuropathy - please leave a comment here.