Introduction

The signs and symptoms experienced during the course of CNSV (including onset) are many and varied, and the proportions given here for these signs and symptoms are intended only to give a general overview of their relative frequency among patients. These proportions are based on the literature (see sources below).

Please note the following cautions about this list and description:

• The terms used in the literature are not completely comparable. We have grouped together similar manifestations. Never the less, there are some overlaps among these due to differing terminology and groupings.
  
  
• Some of the cases in the literature are from before the general use of corticosteriods and cyclophasphamide (and other approaches) to treat CNSV.
  
  
• This list covers a wide variety of people and a wide variety of individual circumstances. Diagnosis may have been prompt or delayed. Treatment may have begun earlier or later and been more or less effective. Keep in mind that if untreated, CNSV is invariably fatal, and early diagnosis and prompt treatment are critical to bringing the disease under control (i.e., to halt ongoing inflammation and to prevent further damage).
  
  
• Some manifestations might be attributable to the treatments and not to the disease.
  
  

Thus, you should understand: (1) that this list might exagerate the extent of some signs and symptoms and (2) that the following proportions are not relevant to any individual person. Clearly, not everyone will have every symptom, and each person will have different combinations of symptoms.

Neurologists and others may use general terms such as diffuse cerebral signs or focal cerebral signs to describe their observations of people with CNSV. "Cerebral signs" are indications of a brain-related, pathological condition -- an inability of the brain to function normally. "Diffuse cerebral signs" result from widely distributed problems in the brain and can be caused by general inflamation, for example. "Focal cerebral signs" are caused by a localized, structural problem that is limited to one specific area. These signs can be the result of space-occupying lesions, for example. Diffuse cerebral signs are experienced by about 80 percent of people with CNSV. Focal cerebral signs are experienced by about 70 percent of people with CNSV.

During the course of CNSV, a majority of people with CNSV experience mental changes, headache, general confusion and/or paralysis/muscle weakness. Compared to initial onset, considerably increased proportions of people with CNSV experience mental changes, general confusion, paralysis/muscle weakness, visual problems, dysphasia, coma, seizure, spinal cord signs and depressed level of consciousness. Never the less, the course of CNSV is different for every person, depending on the list of cautions noted above and on which part of the brain is affected and the amount of damage occurring there.

Mental Changes (About 70%)

During the course of CNSV, most people experience altered mentation and changes in their intellectual functioning and/or ability to reason. Thoughts may have become jumbled; normally easy mental tasks may have become difficult or iffy; the ability to reason and/or make decisions may have become clouded. The ability to conceive of ideas and to make judgements -- even the ability to carry out every day tasks -- may have become shaky. Not every thinking activity is affected, and the difficulties may come and go and be mild at first. (See also: confusion">general confusion, dysphasia and aphasia)

Headache (About 65%)

Most people with CNSV have agonizing, persistant, strong, painful, sometimes prolonged headaches. "Headache," in fact, is not a good word, because these are NOT like ordinary headaches no matter how painful. These headaches seem to come in a variety of forms. There may be "sensations," pressure or shock-like pains. The sensations may seem to be inside the skull, and they may feel like very unpleasant creaping or crawling on or under the scalp. Also in the sensations category, it may feel like the head is on fire, and the pain may flow from one place to another and give the sensation that it will explode. There may be pressure or throbbing inside the skull or a tightness around the skull like a very tight hat band. There may be pain at the temples. There may be severe, shooting, stabbing, darting pain that might be like electric shocks. Medication doesn't offer much relief. While exeriencing theses "headaches," people with CNSV may find that noise and light make them worse. After experiencing these "headaches," people with CNSV may be left sweating, shaken and weak.

General Confusion (About 65%)

Most people with CNSV experience general confusion, not in terms of thinking per se but in terms of orientation to their environment. For example, they may become disoriented in regard to time, place or person, even as they are aware of that disorientation. They may forget what they were doing or were about to do. When out of the house, they may be confused by activity around them, bright or moving lights and/or loud noise. In addition, there may be memory problems or even dementia or personality changes. (See also: mental changes, dysphasia, aphasia and depressed level of consciousness.)

Paralysis and/or Muscle Weakness (About 65%)

Most people with CNSV experience partial or more serious paralysis and lose the ability to use some body part. Most have hemiparesis (50%) -- paralysis affecting only one side of the body. Many experience focal weakness (30%) -- a loss of movement or sensation in one specific area. Some may have developed ataxia (25%) -- a partial or complete failure of muscular coordination, resulting in irregular movements of the body. Some may have developed paraparesis (20%) -- slowy developing muscle weakness, spasms and stiffness of the legs or lower body. Some may have "gait disorder" (13%) or gait ataxia (6%) -- walking that is clumsy or uncoordinated. A few people may have weakness in the facial nerve (cranial nerve VII) not as a part of hemiparesis (9%) causing a decreased ability to move facial muscles, such as to wrinkle the forehead. A few may experience quadriparesis (6%) -- weakness of all four limbs. A few may have parkinsonism (5%) -- a group of neurological disorders characterised by tremor and muscular rigidity. A few may experience pain in the limbs (3%). Finally, a few may experience internuclear ophthalmoplegia (3%) -- paralysis of eye muscles due to nerve damage.

Visual Problems (About 40%)

Many people have vision problems associated with CNSV, and these may show up in a variety of ways. Some have nonspecific (22%) complaints. Some may have papilledema (20%) -- abnormally large amounts of fluid in the optic disk (papilla), most commonly due to increased intracranial pressure. Some may have choked discs (18%). Some may experience nystigmus (10%) -- uncontrolable, rapid movement of the eyeball. Some may have a visual field cut (10%) -- loss of vision in some parts of the visual field. Some may have a disorder of the occulomotor nerves (10%) where a lesion may result in outward deviation of the eyeball, drooping eyelid, double vision or a dilated pupil. A few may have visual changes (9%) such as double vision, blurred vision or even blindness from a lesion in the brain. A few may have optic atrophy (6%) where vision information is not transmitted from the eye to the brain. A few may experience impaired visual acuity (4%) -- reduced ability to detect visual details. A few may have changes in the retina (3%). (See also hemisensory loss and cranial nerve.)

Dysphasia (About 35%)

Many people with CNSV have dysphasia -- a language/speech impairment causing an inability to speak words which one has in mind or to think of correct words, or an inability to understand spoken or written words, or slurred speech. (This is a symptom common to "injuries" of the dominant cerebral hemisphere, particularly the frontal, temporal and parietal lobes.) (See also: aphasia).

Coma (About 35%)

Many people with CNSV go into a coma -- a deep and prolonged unconsciousness where someone cannot be aroused even by strong or painful stimuli, cannot speak and does not respond to command.

Seizure (About 30%)

Some people have one or more seizures during the course of CNSV. In its mild form, a seizure can be simply a brief "loss of contact" or a few moments of what appears to be daydreaming. A seizure also can be an "attack" or convulsion -- a sudden, violent, uncontrollable contraction of a group of muscles.

Depressed Level of Consciousness (About 25%)

Some people experience a depressed level of consciousness. That is, they have a reduced, limited or fluctuating sense of awareness of themselves and of the environment around them. (See also: loss of consciousness.)

Myelopathy (About 20%)

Some people with CNSV have myelopathy -- a general term for any disease affecting the spinal cord which is covered by myelin, a layer around nerves that helps transmit impulses.

Spinal Cord Signs (About 20%)

Some people with CNSV show spinal cord signs -- a general term indicating problems in the spinal cord.

Aphasia (About 20%)

Some people with CNSV experience aphasia -- a disturbance in language caused by a lesion in the brain. As a result, they may have difficulty with speaking, reading or writing or with understanding the written or spoken word. Word finding when speaking or writing and comprehension while listening or reading may be compromised. (See also: dysphasia">dysphasia.)

Fever (About 15%)

Some people have a fever during the course of CNSV.

Systemic Symptoms (About 15%)

With CNSV, some people have systemic symptoms -- that is, generalized symptoms affecting the body as a whole.

Meningeal Signs (About 15%)

Some people with CNSV show meningeal signs -- a general term indicating problems in the meninges, the surrounding membranes of the brain and spinal cord.

Hemisensory Loss (About 15%)

Some people have hemisensory loss -- the loss of sensations on only one side of the body. (See also Visual problems and cranial nerve.)

Psychiatric (About 10%)

A few people have psychiatric indications that would call for the diagnosis, treatment and/or prevention of mental illness.

Weight Loss (About 10%)

A few people experience weight loss.

Loss of Consciousness (About 5%)

A few people experience loss of consciousness. (See also: depressed level of consciousness.)

TIA-Like Episodes (About 5%)

A few people have TIA-like episodes. A transient ischaemic attack (TIA) is a "mini-stroke" caused by a temporary disturbance of the blood supply to an area of the brain. This may cause temporary paralysis, numbness, speech difficulty or other neurologic symptoms. TIAs start suddenly and recovery comes in less than 24 hours, usually less than 1 hour.

Vertigo (About 5%)

A few people experience vertigo -- the illusion of movement. They may feel that the external world is circling them or that they are circling through space.

Lumbosacral Radiculopathy (About 5%)

A few people may have lumbosacral radiculopathy --a herniated, slipped or ruptured disk.

Cranial Nerve VIII (About 5%)

A few people may have problems with cranial nerve VIII -- the nerve responsible for hearing and balance. There can be problems with deafness, tinnitus, dizziness, vertigo">vertigo and vomiting.

Other Symptoms

During the course of CNSV, a few people experience other focal disorders HIF, lower cranial nerves weaknesses, hemiataxia, "cortical" sensory loss, cauda equina syndrome and fasciculations (spontaneous, small twitches).