Central Nervous System Manifestations In Scleroderma PDF Print E-mail
Saturday, 26 March 2011 21:24
taken from http://www.flickr.com/photos/78572993@N00/2226696853/sizes/m/in/photostream/ via creative commonsWe at the Scleroderma Care Foundation believe that knowledge is indeed, power. It was this belief that became the primary motivation for the creation of our website. It was meant to empower all patients to take control of the disease through news and informative articles, and tips and tricks for a living a healthier life in spite of the disease.

Very recently, it was brought to our attention that a couple of our patients may be suffering from what we suspect and our indicates, may be some derivative of Transient Ischemic Attack (TIA) - an often rare central nervous system manifestation of progressive systemic sclerosis or Scleroderma. Today, we've put together some information on this and other similar manifestations, in hopes that it may assist those that may be currently affected by it, or may suspect that a similiar condition may be affecting them.

Do remember that we would encourage each of you armed with the right information, to visit your own doctor or specialist for proper diagnosis and treatment.

As we are already aware, Systemic Sclerosis (Scleroderma) is a rare disorder resulting from the excessive production and deposition of collagen in the skin, blood vessels, and other organs. According to the Current Opinion in Rheumatology, neurologic involvement is rare, with myopathy and cranial neuropathies being the most frequently reported manifestations. Brachial plexopathy, lumbosacral radiculopathy, and polyneuropathy have also been reported. Central nervous system manifestations are even more rare and may be due to hypertension, renal or pulmonary dysfunction caused by Scleroderma, or primary vascular changes. These manifestations include Encephalopathy, Aphasia, Dementia, Psychosis, Anxiety disorder, Grand mal seizures, and Transient Ischemic Attacks.

According to MedicineNet.com, Encephalopathy is a term that means brain disease, damage, or malfunction. Encephalopathy can present a very broad spectrum of symptoms that range from mild, such as some memory loss or subtle personality changes, to severe, such as dementia, seizures, coma, or death. In general, encephalopathy is manifested by an altered mental state that is sometimes accompanied by physical manifestations (for example, poor coordination of limb movements).

The term encephalopathy is very broad and in most cases, is preceded by various terms that describe the reason, cause, or special conditions of the patient that leads to brain malfunction. For example, anoxic encephalopathy means brain damage due to lack of oxygen, and hepatic encephalopathy means brain malfunction due to liver disease. Additionally, some other terms either describe body conditions or syndromes that lead to a specific set of brain malfunctions. Examples of these are metabolic encephalopathy and Wernicke's encephalopathy (Wernicke's syndrome). There are over 150 different terms that modify or precede "encephalopathy" in the medical literature.

According to the National Institute of Neurological Disorders and Stroke (2010), Aphasia is a neurological disorder caused by damage to the portions of the brain that are responsible for language. Primary signs of the disorder include difficulty in expressing oneself when speaking, trouble understanding speech, and difficulty with reading and writing. Aphasia is not a disease, but a symptom of brain damage. Most commonly seen in adults who have suffered a stroke, aphasia can also result from a brain tumor, infection, head injury, or dementia that damages the brain. It is estimated that about 1 million people in the United States today suffer from aphasia. The type and severity of language dysfunction depends on the precise location and extent of the damaged brain tissue.

Generally, aphasia can be divided into four broad categories:
  • Expressive aphasia involves difficulty in conveying thoughts through speech or writing. The patient knows what he wants to say, but cannot find the words he needs.
  • Receptive aphasia involves difficulty understanding spoken or written language. The patient hears the voice or sees the print but cannot make sense of the words.
  • Patients with anomic or amnesia aphasia, the least severe form of aphasia, have difficulty in using the correct names for particular objects, people, places, or events.
  • Global aphasia results from severe and extensive damage to the language areas of the brain. Patients lose almost all language function, both comprehension and expression. They cannot speak or understand speech, nor can they read or write.

WedMD notes that Dementia is the loss of mental functions -- such as thinking, memory, and reasoning -- that is severe enough to interfere with a person's daily functioning. Dementia is not a disease itself, but rather a group of symptoms that are caused by various diseases or conditions. Symptoms can also include changes in personality, mood, and behavior. In some cases, the dementia can be treated and cured because the cause is treatable. Examples of this include dementia caused by substance abuse (illicit drugs and alcohol), combinations of prescription medications, and hormone or vitamin imbalances. In some cases, although the person may appear to have dementia, a severe depression can be causing the symptoms. This is known as pseudo-dementia (false dementia) and is highly treatable. In most cases, however, a true dementia cannot be cured.

Dementia develops when the parts of the brain that are involved with learning, memory, decision-making, and language are affected by one or more of a variety of infections or diseases. The most common cause of dementia is Alzheimer's disease, but there are as many as 50 other known causes. Most of these causes are very rare. Because some causes of dementia can be cured or partially treated, it is very important that your doctor is thorough when making the diagnosis, so as not to miss potentially treatable conditions. The frequency of "treatable" causes of dementia is believed to be about 20%.

Psychosis is a serious but treatable medical condition that reflects a disturbance in brain functioning. A person with psychosis experiences some loss of contact with reality, characterized by changes in their way of thinking, believing, perceiving and/or behaving. For the person experiencing psychosis, the condition can be very disorienting and distressing. Without effective treatment, psychosis can overwhelm the lives of individuals and families. Psychosis is a medical condition that affects the brain. It can be treated.

A person with psychosis may:
  • experience confused thoughts
  • feel their thoughts have sped up or slowed down
  • feel preoccupied with unusual ideas
  • believe that others can manipulate their thoughts; or that they can manipulate the thoughts of others
  • perceive voices or visions that no one else can hear or see
  • feel 'changed' in some way
  • act differently than they usually would
Sometimes psychosis emerges gradually over time, so that in the early stages symptoms might be dismissed or ignored. Other times, symptoms appear suddenly and are very obvious to the individual and those around them. Symptoms vary from person to person and can change over time. The initial experience of psychotic symptoms is known as the 'first episode' of psychosis. It is important to pay attention to possible symptoms and seek help early.

Anxiety Disorders
There are many types of anxiety disorders that include panic disorder, obsessive compulsive disorder, post traumatic stress disorder, social anxiety disorder, specific phobias, and generalized anxiety disorder.

Anxiety is a normal human emotion that everyone experiences at times. Many people feel anxious, or nervous, when faced with a problem at work, before taking a test, or making an important decision. Anxiety disorders, however, are different. They can cause such distress that it interferes with a person's ability to lead a normal life. An anxiety disorder is a serious mental illness. For people with anxiety disorders, worry and fear are constant and overwhelming, and can be crippling.

Transient Ischemic Attacks
A transient ischemic attack (TIA) is an episode in which a person has stroke -like symptoms for up to 1-2 hours. It is often considered a warning sign that a true stroke may happen in the future if something is not done to prevent it. A transient ischemic attack (TIA) is caused by temporary disturbance of blood supply to an area of the brain, which results in a sudden, brief decrease in brain function. (A decrease in brain function is called a neurologic deficit.)

A TIA is different than a stroke. Unlike from a stroke, a TIA does not cause brain tissue to die. The symptoms of TIAs do not last as long as a stroke and do not show changes on CT or MRI scans. (Strokes usually show changes on such tests.)

The temporary loss of blood flow to the brain can be caused by:
  • Blood clot within an artery of the brain
  • Blood clot that travels to the brain from somewhere else in the body (for example, the heart)
  • Injury to blood vessels
  • Narrowing of a blood vessel in the brain or leading to the brain

For instance, the temporary disruption in blood flow could be due to a blood clot that occurs and then dissolves. The blockage breaks up quickly and dissolves.

Less common causes of TIA include:
  • An irregular heart rhythm called atrial fibrillation
  • Certain blood disorders, including polycythemia, sickle cell anemia, and syndromes where the blood is very thick
  • Conditions that cause blood vessels problems such as fibromuscular dysplasia, systemic lupus erythematosus, and syphilis
  • Inflammation of the arteries such as arteritis, polyarteritis, and granulomatous angiitis
  • Spasm of the small arteries in the brain
Atherosclerosis ("hardening of the arteries") is a condition where fatty deposits occur on the inner lining of the arteries. This condition dramatically increases the risk for both TIAs and stroke. Approximately 80-90% of people who have a stroke due to atherosclerosis had a TIA episode before. Other risks for TIA include high blood pressure, heart disease, migraine headaches, smoking, diabetes, and increasing age.


According to one paper, Neuropathological data is scarce in Systemic Sclerosis and fail to demonstrate primary changes in the brains of such patients. Literature addressing neurologic involvement in Scleroderma principally consists of case series and case reports. It includes the following categories, listed in approximate order of frequency:
  • Cranial, entrapment, peripheral, cutaneous, autonomic neuropathies
  • Myopathy
  • Central nervous system involvement, including headache, seizures, stroke, vascular disease, radiculopathy, and myelopathy

In general, however, central nervous system (CNS) involvement due to Scleroderma is rare, but complications of other organ damage may have neurologic manifestations. Examples include renal failure, uncontrolled hypertension, and heart or respiratory failure. For more information on these manifestations and more, we strongly encourage to visit some of the sources and links located below. For those of you that have an account on our site, do login to download a couple case reports and research papers we were able to find.

Chin and Latov (2011), "Central Nervous System Manifestations of Rheumatologic Diseases: Progressive Systemic Sclerosis (Scleroderma)", Medscape Today; original article can be viewed here.
National Institute of Neurological Disorders and Stroke (2010), "NINDS Myopathy Information Page"; original article can be viewed here.
MedicineNet.com (2011), "Encephalopathy"; original article can be viewed here.
National Institute of Neurological Disorders and Stroke (2010), "NINDS Aphasia Information Page"; original article can be viewed here.

Canadian Mental Health Association (2011), "Psychosis"; original article can be found here.
Canadian Mental Health Association (2011), "Anxiety Disorders"; original article can be found here.
WebMD.com (2011), "Anxiety Disorders"; original artlice can be viewed here.
Piecyk, M. and Schur, P. (2010), "Neuromuscular manifestations of systemic sclerosis (scleroderma)"; original article can be viewed here.
Hoch, B. and Zieve, D. (2010), "Stroke"; original article can be viewed here.
Hoch, B. and Zieve, D. (2008), "Dementia"; original article can be viewed here.
WebMD.com (2011), "Alzheimer's Disease and Other Forms of Dementia"; original artlice can be viewed here.
Zieve, D. (2010), "Transient ischemic attack"; original article can be viewed here.
National Institute of Neurological Disorders and Stroke (2010), "NINDS Transient Ischemic Attack Information Page"; original article can be viewed here.

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+2 #2 Kelly Fuller 2014-04-12 13:50
I have been being treated for RA and PsA with no relief of symptoms and seem to be in constant flare all the while turning into the Crypt Keeper at an alarming rate but seem to be having seizure like activity with strong muscle contractions that last several seconds to several minutes. I am aware during them but can't respond. They are unpredictable but I do feel them coming on and they seem to have floating effecting. I have been so much more unable to deal with any stress at all. I try to talk and and can't find words, I've lost an inch in height in the past yr and am dealing with the RA/PSA dx.so of course there is the anxiety to over that I am trying to accept. Some say well if you would just relax. Well I think this sounds so much like what is going on with me but I don't know how to get a DR to listen to me. They will not listen to matter how much I beg or plead or tell them the same symptoms over and over..Yesterday was in ER for these spells and was sent home DX of spasticity. Last month they had me inpatient of migraine. Im still having daily migraine and the seizure like probs with BP out of control. How can I get tested? Do they do blood work and is it a specialized or sent out test? What type of DR? I have Rhemy and Neuro.? Nobody will listen, Im scared and just so frustrated.
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+1 #1 Dianne Kinney 2012-04-23 02:18
Thank you , Thank you, Thank you.
I love finding out all the medical research etc. Your effort in posting this info is very appreciated.
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