Spinal cord lesions in MS

Multiple sclerosis is a demyelinating disease of the central nervous system (demyelinating because the disease is characterized by the loss of the myelin sheath around the axons of the nerve cells). As I have stated in my previous posts on MS (see http://braindiseases.info for all the previous posts on MS), the disease is characterized by plaques which are disseminated in space and time.

Most of these plaques (demyelinating lesions of MS) are seen in the brain but a few patients have what is loosely called spinal MS or rather MS in which the plaques are more commonly seen in the spinal cord (remember the spinal cord is a part of the central nervous system). These patients with spinal MS present with slightly different clinical signs and symptoms. They may present with what is called transverse myelitis (this is an involvement of the spinal cord usually at the cervical or thoracic level). Transverse myelitis can be devastating because all the descending motor fibers from the brain and the ascending sensory tracks are packed in the small diameter of the spinal cord. So any involvement of the spinal cord has the potential to affect all these tracks. Depending upon the level of cord involvement patients may have either weakness of just the legs (paraparesis or paraplegia) or all the four limbs may be involved (quadriparesis or quadriplegia). Usually the bladder and bowel are involved too and patients may have complaints of urinary incontinence. Sexual dysfunction is also commonly reported (erectile dysfunction in males, see my previous post on it).

As the involvement of the brain is less, these patients are relatively well preserved cognitively and may not have prominent cerebellar findings.

Spinal involvement in MS is treated in much the same way as other forms of MS. Your doctor may use a course of intravenous corticosteroids if you present to the hospital with acute transverse myelitis. Immuno modulating drugs like interferons may later be prescribed.

Personal Regards,

Nitin Sethi, MD

Bells Palsy: what to expect

Let us talk a little about Bells palsy a relatively common neurological condition affecting the function of the seventh cranial nerve (that is the facial nerve).  The muscles of the face are suplied by the facial nerve. It is this nerve which helps you to smile, frown, wrinkle up your forehead, purse your lips and all other facial expressions. The facial nerve is also the nerve which supplies taste sensation to the anterior part of the tongue. It suppiles a muscle called stapedius in the middle ear which helps in damping loud sounds and also supplies the lacrimal gland (helps in tear function).

In Bells palsy patients develop weakness of all the muscles of one half of the face (they cannot close their eye on that side, have a crooked smile as the face gets pulled to the normal healthy side when they attempt to smile, cannot puff their cheeks or purse their lips to whistle). Depending upon the site of involvement of facial nerve, they may also be unable to tear (complaint of dry eye), unable to taste food or complain of excessive loud sounds in the ear on the side of the facial nerve palsy.

In Bells palsy there is inflammation of the facial nerve somewhere along its course from the brainstem to the muscles of the face. In Bells palsy, this inflammation is idiopathic (no definite cause for inflammation is found) though inflammation by the herpes group of viruses has been implicated in its etiology. Some patients have developed Bells palsy after been exposed to cold air (this has never been proved though). Bells palsy is not life threatening but can be quite socially disabling especially if severe (you can imagine how socially disruptive it might be to have one half of your face paralysed and be unable to smile or emote with your face).

Diagnosis and management of Bells palsy: the diagnosis of Bells palsy is clinical (can be made by a clinical examination by a neurologist). In the typical case no further investigations are warranted, though in some cases if the history is atypical (slow onset with addition neurological findings) your doctor may order an MRI study of the brain. 

Most of the patients recover spontaneously from Bells palsy. All that we advise them is to cover the eye at night with an eye patch and wear sunglasses when they go out during the day (this is to protect the eye from keratitis as the eye does not shut). If you present acutely to your doctor with Bells palsy, he may recommend a course of steroids and acyclovir (antiviral drug with efficacy against herpes virus). It is thought that this hastens recovery though some studies have shown that the recovery is the same whether or not acyclovir is used or not).

Your doctor shall also recommend facial exercises (just as you undergo physical therapy if you have a stroke, in the same way we want to encourage facial exercises to hasten recovery). Facial exercises are easy to do and I usually recommend my patients to stand in front of the mirror and attempt to smile, frown, pout and whistle. I ask them to do this for at least 10 mins two times a day. Massaging the face has not been shown to be beneficial. In case the recovery is slow or incomplete your doctor might order a nerve conduction study of the facial nerve to assess the degree of damage to the nerve.

As I stated earlier most patients have a good recovery. At times when the facial nerve regenerates, it regenerates in an arbitary fashion. Patients develop crocodile tears (they start tearing when attempting to eat) or have synkinesis (all the muscles of the face contract at once when attempting to smile that is the fine control of individual facial muscles is lost). If you have these problems you should contact your doctor and ask for advise.

I hope this short post on Bells palsy shall be helpful to some of you.

Personal Regards,

Nitin Sethi, MD

Infantile spasms and hypsarrhythmia: what do we know?

One of my readers emailed me and asked me to talk a little about infantile spasms and hypsarrhythmia as he has a niece who has been diagnosed with this condition. So in this post we shall talk about the same.

First and foremost the term hypsarrhythmia does not refer to a disease, it actually refers to a characteristic EEG pattern seen in some infants who have infantile spasms (their EEG shows some special features and is characteristic for their disorder). So what do we mean by infantile spasms. Infantile spasms as the name suggests are spasms which occur in infants (by infants I mean less than one year of age). These are special kind of seizures in which the infant is noted to have spasm like movements. It is hard to describe what spasms look like clinically but they can be of 2 types: extensor spasms and flexor spasms (the infant is noted to have sudden abduction of the arms and legs, a good example would be to imagine what you would do if some one suddenly frightens you: you shall suddenly jerk and abduct your arms and legs).

In infantile spasms, infants have flexor and extensor spasms. They usually occur in clusters and sometimes an infant may have as many as 15-20 spasms in 5 mins. These spasms represent seizures and when you do an EEG on these infants you do find characteristic EEG findings suggestive of seizures. This now brings us to the term hypsarrhythmia. As I stated earlier this refers to the EEG of a child with infantile spasms (the EEG is disorganized, of very high amplitude and shows multi-focal epileptiform activity. By multi-focal I mean, that there are many spots/ areas in the brain which shows signs of epileptogenicity). Infants who have infantile spasms may also have other types of seizures as they grow up. They may have seizures characterized by sudden jerks (we call these myoclonic jerks) and other more typical seizures where-in they have jerks of the arms and legs.

Etiology/ causes of infantile spasms: an infant may have infantile spasms and no cause may be determined even after a good work up. In that case the condition is referred to as idiopathic infantile spasms (idiopathic meaning for which no cause is determined). Usually though in most infants a cause for infantile spasms can be determined after a thorough work up. Work-up for infantile spasms usually should be carried out in a big center where the doctors have sufficient experience in dealing with these complicated cases. The doctor shall order many tests. Some of them include an EEG (at times the doctor might admit the infant and do a more prolonged EEG test. This is called a video-EEG monitoring test). Other tests which may be carried out include imaging studies of the brain such as an MRI (children and babies usually have to be sedated prior to the MRI test), tests of the blood to rule out any metabolic and storage diseases etc.

Once the diagnosis is secured and the etiology determined, then the question of management arises. Management involves 2 issues, one is the management of the underlying condition which is the cause of the infantile spasms (if the spasms are idiopathic we do not have to deal with this issue), the other is the management of the spasms/ seizures itself.

Infantile spasms usually respond to ACTH (adrenocorticotrophic hormone), a type of steroid preparation. It has been seen that when infants are started on ACTH, their spasms may completely stop and their EEG may also normalize (that is the hypsarrhythmia pattern goes away). There are other drugs which can be used too and your doctor shall help you in deciding the best option. It has been noticed that in some infants as the ACTH is stopped the spasms come back.

It is important that infantile spasms be detected and treated in time because ongoing spasms effect the cognitive development of the child and may lead to developmental arrest.

I hope this shall be helpful to some of you. If you seek more information, please do let me know.

Personal Regards,

Nitin Sethi, MD