The very basic answer is that cerebrovascular disease can cause
epilepsy, especially in people over the age of 65. Strokes, high blood
pressure, smoking, malformed blood vessels of the brain, and blood
clots in the brain can cause a hemorrhage that can irritate brain
tissue and nerves, leading to seizures.
?Stroke is one of the most common causes of epilepsy among seniors and
adults in general. o8% - 13% of stroke survivors have a seizure in the
hours to years following a stroke.
Of these, more than 50% are early onset seizures
(within 1 week but most often within the first 24 hours of the stroke).
o Epilepsy develops in 3 - 4% of stroke patients.
Early onset seizures after stroke are common and have little affect on
the outcome. (Up to 33% developed epilepsy.)
Late onset seizures are less common, but are associated with recurrent seizures.
(50% developed epilepsy.)
o Seizures are more common after a haemorrhagic stroke than an ischemic stroke.
?The brain is the most complex organ in the body, needing plentiful
amounts of oxygen to stay in good working order. Large blood vessels
bring oxygen-bearing blood to the brain and a web of tiny blood
vessels distribute it to every area.
Vascular disease describes a condition where the blood vessels are
damaged and the supply of oxygen is at risk. If the oxygen supply
fails in the brain, brain cells are likely to die, leading to strokes
and possible vascular dementia. This disease is sometimes described as
cerebral infarction. The effects may be:
? Strokes, which can be fatal but which may otherwise lead to physical
disability and mental confusion or both.
? Multi-infarct dementia (MID), caused by a series of very small
strokes in the brain
? A rare, slowly developing dementia caused by diseased blood vessels
deep in the brain, known as subcortical vascular dementia or
Several factors increase the risk of vascular disease. The most
significant are high blood pressure and smoking. Cigarette smoking is
implicated in about a third of strokes.
? Stopping smoking, losing excess weight, taking exercise and reducing
levels of salt in food are all ways to help prevent vascular disease
and therefore strokes or MID.
? People at high risk of strokes, including those who have had a mild
warning stroke (a transient ischaemic attack), can reduce the risks
through blood thinning drugs including aspirin.
Is treatment possible?
Brain cells which have been destroyed cannot be repaired, and so the
damage done to the brain by a stroke is permanent. However, the brain
can sometimes find ways to function around damaged areas, and
confusion after a stroke does sometimes improve.
People with vascular dementia should be offered a skilled
multidisciplinary assessment of their condition through referral to a
specialist. This service may be part of the general stroke service or
the psychogeriatric service.
? People who have had strokes do better when seen by specialist
multidisciplinary teams for treatment and rehabilitation focused on
restoring as much mental or physical ability as possible.
? Specialist doctors can ensure accurate diagnosis so that any
underlying disease, such as hypertension or diabetes, is treated.
? Speech and language therapists help someone make the best use of
? An occupational therapist specialising in mental health can help
someone cope better in daily life and be safer in the home.
? In severe cases nursing care may be needed.
?Secondary brain injury refers to the changes that evolve over a
period of time (from hours to days) after the primary brain injury. It
includes an entire cascade of cellular, chemical, tissue, or blood
vessel changes in the brain that contribute to further destruction of
?Epilepsy occurs in 2 to 5 percent of all people who sustain brain
injury, but it is much more common with severe or penetrating
injuries. While most seizures occur immediately after the injury, or
within the first year, it is also possible for epilepsy to surface
years later. Epilepsy includes both major or generalized seizures and
minor or partial seizures.?
?The main risk factors include age, genetical factors, diabetes,
abnormalities of lipid metabolism, cigarette smoking, hypertension,
and obesity. It affects both extracranial and intracranial blood
vessels. Patients may present with transient ischaemic attacks and
these patients have a high risk of stroke. Platelet-fibrin emboli or
cholesterol crystals can sometimes be seen in the retina on
fundoscopy. Strokes are frequently embolic in origin and the emboli
often arise from extracranial arteries and from the heart.?
?Raised pressure exposes smaller blood vessels to excessive pressure
and blood flow which, following breakdown of auto regulation, results
in an increase in blood flow proportional to the rise in blood
pressure. High pressure results in disruption of the blood-brain
barrier with transendothelial transport of plasma constituents across
the vessel wall (Figure 6). The deposition of plasma proteins within
the wall of small blood vessels results in progressive disruption of
smooth muscle cells, fibrosis (Figure 7), and eventually fibrinoid
?Cerebrovascular disease is any disease affecting an artery within
the brain, or supplying blood to the brain. The most common is
atherosclerosis, where plaques (fatty deposits) form, leading to
narrowing of the arteries. There may also be a defect or weakness in a
blood vessel in the brain which can cause an aneurysm (ballooning of
Cerebrovascular disease makes it more likely that a stroke will occur,
when there is a sudden blockage or rupture of a blood vessel within
the brain. Blockage may be due to a blood clot forming in the cerebral
arteries (a thrombosis) or by a fragment of material (blood clot,
piece of tissue, cholesterol or various other substances) travelling
in the blood stream (an embolism).?
?Any injury to the head or brain usually results in some damage to
blood vessels in the brain. While the body usually quickly repairs
damage to small blood vessels, an injury to larger vessels can result
in serious complications. Damage to a major artery supplying blood to
the brain can cause a stroke in one of two ways: 1) bleeding from an
artery (called a hemorrhagic stroke), or 2) a blood clot that forms in
an injured artery. When a clot forms in a major artery it can block
blood flow, depriving the area that the artery supplies with blood of
needed oxygen and nutrients (known as an ischemic stroke). Symptoms of
a blood clot in the head include headache, vomiting, seizures,
paralysis on one side of the body, and semi-consciousness.?
?Can other conditions that affect the brain cause epilepsy?
Epilepsy is far more common in those who have other conditions which
affect the brain. These conditions include cerebral palsy, learning
difficulties, cerebrovascular disease (stroke) and dementia. All of
these conditions have different causes, but the common link between
them is a brain abnormality, i.e. something has gone wrong in the
brain or the brain has been damaged in some way. It is the brain
abnormality, whatever its cause, which is responsible for causing all
these problems, including any epilepsy. However, none of these
conditions actually causes epilepsy, and neither does epilepsy cause
cerebral palsy, learning difficulties, behaviour problems and so on.?
?Cerebral vascular disease resulting in stroke or intracranial
hemorrhage represents an important cause of seizures especially in the
elderly. Cerebral infarction in the older population (> 50 years of
age) has been found to be the cause of new-onset seizures in
approximately 20% of cases. (13) Seizures may complicate subarachnoid
hemorrhage in 10 - 20% of cases and are probably more common in
patients that harbor a peri-sylvian or temporal lobe hematoma. (14)
Intracerebral hematomas, especially of the lobar type, are associated
with seizures in about 28% of cases whereas deep basal ganglia
hematomas have a much lower incidence. (15)
Craniocerebral trauma has also been associated with approximately a
20% risk of post-traumatic epilepsy. (16) Diffuse closed head injury
has a lower incidence than compound fractures or penetrating cerebral
injury or closed head injury associated with intracranial hematomas.
Mechanisms of Epilepsy
Although it is clear that seizures can be associated with structural
cerebral lesions it is not known by what mechanisms these seizures
occur. It is generally acknowledged that the seizures do not arise
from the lesion itself but rather from the irritated cortex
immediately adjacent to the lesion. On occasion, seizures may arise
from within the border zone of an infiltrative lesion such as an
oligodendroglioma or astrocytoma.?
?Exact mechanisms of epileptogenicity are unknown although a number of
electrophysiologic and pathophysiologic theories have been proposed.
These include changes in altered neurotransmitter levels (GABA and
somatostatin), free radical formation and altered second messenger
physiology. (17) Morphological changes have also been identified such
as alterations in vascular supply, neuronal cell loss, glial
proliferation and subtle subcortical disconnections. (18) In the case
of AVMs and cavernous angiomas and intracerebral hemorrhage, it is
suspected that the breakdown products of the blood deposit ferric ions
into the surrounding cortex which is a known epileptogenic substance.?
?Epilepsy is often, but not always, the result of an underlying
brain disease. Any type of brain disease can cause epilepsy, but not
all people with the same brain disease will have epilepsy. In view of
the fact that only a proportion of people who have a brain disease
experience seizures as a symptom of that disease, it is suspected that
those who do have such symptomatic seizures are more vulnerable due to
? There are still many people for whom the cause of their epilepsy
cannot, as yet, be identified. In such cases, the theory most commonly
accepted is that this epilepsy is the result of an imbalance of
certain chemicals in the brain (especially chemical messengers known
as neurotransmitters) causing them to have a low convulsive threshold.
? Children and adolescents are more likely to have epilepsy of unknown
or genetic origin. The older the patient, the more likely it is that
the cause is an underlying brain disease, such as a brain tumour or
cerebrovascular disease, or is the result of head injury.
? Trauma and brain infection can cause epilepsy at any age, and as
mentioned previously may account for a higher incidence of epilepsy in
developing countries. For example, a common cause in Latin America is
neurocysticercosis cysts on the brain caused by tapeworm infection,
while in Africa, malaria and meningitis are common causes, and in
India neurocysticercosis and tuberculosis often lead to epilepsy.
? Febrile illness of any kind can trigger seizures in young children.
About 3% of children who have febrile convulsions go on to develop
epilepsy in later life.
?The term stroke refers to a neurologic deficit with sudden or
rapid onset due to hemorrhagic or ischemic cerebrovascular disease,
which lasts 24 hours or more. A deficit due to ischemia that lasts
less than 24 hours is a transient ischemic attack (TIA).
Historical references for stroke as a cause of seizures or epilepsy
date back to Hippocrates, but it was not until Hughlings Jackson in
1864 that brain damage from stroke was clearly defined as a cause of
Subsequent studies have confirmed that cerebrovascular disease is the
most common documented cause of seizures in the elderly, accounting
for 22?69% of seizures in this age group.3?5 As the population ages,
cerebrovascular disease will account for an increasingly high
percentage of total cases of seizures and epilepsy.
An additional relationship between seizures and vascular disease
concerns differential diagnosis: In some cases, it is difficult to
determine whether the cause of transient neurologic dysfunction is
ischemia (TIA) or seizure and, if from a seizure, whether the seizure
itself results from a new vascular insult. These distinctions have
major implications for treatment.? Please continue reading this page
for further information.
?If you put a drug into the circulating blood, it'll leave the
blood vessels wherever they are in the body, and diffuse into the
surrounding tissues - everywhere, that is, except in the brain. The
blood vessels are different in the brain.?
Regarding cerebral blood vessels: ?But in the brain, there's a
slightly different arrangement where the endothelial cells join up.
The endothelial cells overlap each other at what are called "tight
junctions" - which are almost like thin strips of Velcro where the
flat endothelial cells are very tightly stuck to each other, so even
really small viruses can't sneak through. These "tight junctions" help
make the Blood Brain Barrier.?
?Now this is the big problem with drugs to treat diseases of the
Central Nervous System. 98% of all known potential CNS drugs have a
molecular weight bigger than 500, so they can't cross the Blood Brain
Barrier. The only diseases that we have any sort of half-way
reasonable drugs for are a few CNS diseases such as epilepsy, chronic
pain, schizophrenia and mood disorders - and these are all
?The onset of epilepsy can often be traced to an accident, disease
or medical trauma ? such as a stroke ? that injures your brain or
deprives it of oxygen, often causing a small scar in your brain. In
rare occasions, epilepsy may be caused by a tumor in your brain.
However, in many cases there's no identifiable cause for the disease.?
?Stroke and other diseases that affect your vascular system can lead
to brain damage that may trigger epilepsy. You can take a number of
steps to reduce your risk of these diseases, including limiting your
intake of alcohol, following a healthy diet, managing your weight,
exercising regularly and avoiding cigarettes.
Other epilepsy risk factors include:
? Alzheimer's disease
? Brain infections
? Poisoning from exposure to lead, carbon monoxide and other toxins
?Stroke, heart attacks, and other conditions that affect the blood
supply to the brain (cerebrovascular diseases) can cause epilepsy by
depriving the brain of oxygen. About a third of all new cases of
epilepsy that develop in older people are caused by cerebrovascular
?Seizures are a result of a shift in the normal balance of
excitation and inhibition within the CNS. Given the numerous
properties that control neuronal excitability, it is not surprising
that there are many different ways to perturb this normal balance, and
therefore many different causes of both seizures and epilepsy. Our
understanding of the basic mechanisms involved remains very limited,
and consequently there is not a rigorous, mechanistic-based framework
for organizing all the etiologies. Conceptually, however, three
important clinical observations emphasize how a variety of factors
determine why certain conditions may cause seizures or epilepsy in a
?In practice, it is useful to consider the etiologies of seizures
based on the age of the patient, as age is one of the most important
factors determining both the incidence and likely causes of seizures
or epilepsy. During the neonatal period and early infancy, potential
causes include hypoxic-ischemic encephalopathy, trauma, CNS infection,
congenital CNS abnormalities, and metabolic disorders. Babies born to
mothers using neurotoxic drugs such as cocaine, heroin, or ethanol are
susceptible to drug-withdrawal seizures in the first few days after
delivery. Hypoglycemia and hypocalcemia, which can occur as secondary
complications of perinatal injury, are also causes of seizures early
after delivery. Seizures due to inborn errors of metabolism usually
present once regular feeding begins, typically 2 to 3 days after
birth. Pyridoxine (vitamin B6) deficiency, an important cause of
neonatal seizures, can be effectively treated with pyridoxine
replacement. The idiopathic or inherited forms of benign neonatal
convulsions are also seen during this time period.?
?Cerebrovascular disease may account for approximately 50% of new
cases of epilepsy in patients older than 65. Acute seizures (i.e.,
occurring at the time of the stroke) are seen more often with embolic
rather than hemorrhagic or thrombotic stroke. Chronic seizures
typically appear months to years after the initial event and are
associated with all forms of stroke.?
?Primary angiitis of the central nervous system (PACNS) is a rare,
subacute vasculitis restricted to the vasculature of the CNS.30
Vessels of any size may be affected, but small-vessel vasculitis is
always present.31 Other than constitutional symptoms, such as fever,
malaise, and anorexia, there is no consistent evidence for systemic
disease related to this disorder.
The most common clinical features of the disease include seizure,
stroke, headache, intellectual deterioration, or spinal cord
syndromes.31 Seizures occur in up to 44% of patients.
The pathophysiology of PACNS is not known. It is presumed to have an
immune-mediated basis, based on its response to immune-modulating
drugs. The similarities in histopathologic findings in the brain
between this disorder and others such as sarcoidosis and disseminated
herpes zoster suggest the possibility of an underlying process that is
yet to be identified?
?Researchers in Norway determined that stroke severity measured by
the Scandinavian Stroke Scale is a statistically significant predictor
for epilepsy after stroke. Data shows that more than 20,000 Americans
will develop epilepsy due to stroke each year. This research is
published in the August issue of the journal Epilepsia.?
?In one of the longest follow-up studies performed with data from
almost 500 patients, researchers found that 3.1% of people who
suffered a stroke developed epilepsy. Those who experienced severe
strokes had five-times the risk of developing epilepsy post-stroke
compared to those with less severe strokes. Neither treatment in a
specialized stroke unit, age at onset of stroke, or geographical
location seemed to influence the risk of developing epilepsy after a
stroke in this study.
"It is important to perform further studies to find out whether newer
treatments, such as acute thrombolysis can reduce the frequency of
post-stroke epilepsy," states Morten I. Lossius, Director of the
Department for Education and Research of the National Centre for
Epilepsy in Norway. "It is also important to try to improve the
treatment of stroke patients beyond what today are known as the gold
standards, which apart from thrombolysis, was followed in our study.
New neuroprotective drugs and increased use of thrombolysis may play
an important role in future treatment."
?Sometimes epilepsy is caused by abnormalities in the structure of
the brain. These can be such things as too much spinal fluid
(hydrocephalus), scar tissue, or a tangle of blood vessels (vascular
malformation). Tests that can take pictures of the brain, called
"neuroimaging," can tell doctors whether you have one of these
conditions. These tests are performed to look for the cause of your
seizures (which can be identified for about half of patients) or to
make sure that you don't have some other medical condition.?
?Because of population increases, especially among the elderly,
absolute numbers of strokes and stroke-related deaths have
nevertheless increased. An estimated 750,000 people in the United
States experience stroke each year (recurrent in about 10%), and more
than seven times that figure, approximately 4.4 million, have
experienced stroke before and are at varying risk of developing
?Star-shaped brain cells that are often overlooked by doctors and
scientists as mere support cells appear to play a key role in the
development of epilepsy, researchers say in a study published on-line
August 14 in Nature Medicine. It's one of the first times scientists
have produced firm evidence implicating the cells, known as
astrocytes, in a common human disease.
Scientists found that astrocytes can serve as ground zero in the
brain, setting off a harmful cascade of electrical activity in the
brain by sending out a brain chemical that triggers other brain cells
to fire out of control.?
Please read the entire article for further information:
?By understanding the relationship between potassium and sodium
channels, Cooper and colleagues are working to create new treatments
for neurological diseases based on reestablishing the type of
nerve-cell impulse control seen in unaffected individuals. In fact, a
new drug that acts by increasing the openings of these potassium
channels is now undergoing U.S. and international trials for epilepsy,
and such agents are also being developed for other neurological and
?In people age 65 and older, stroke is the most common known cause,
followed by degenerative conditions such as Alzheimer's disease. Often
seizures do not begin immediately after a person has an injury to the
brain. Instead, a seizure may occur many months later.?
?Epilepsy surgery can benefit patients who have seizures associated
with structural brain abnormalities, such as benign brain tumors,
malformations of blood vessels (including disorders known as
arteriovenous malformations, and cavernous angiomas), and strokes. The
goal of epilepsy surgery is to identify an abnormal area of brain
cortex from which the seizures originate and remove it without causing
any major functional impairment.?
?Epilepsy is not a disease, but a collection of diverse syndromes,
some of which are secondary to other brain derangement?s, and some
which are primary. Any serious injury can lead to epilepsy. Common
ones include: major head trauma, stroke, hemorrhage, infection,
vascular malformations, and benign or malignant tumors. The paroxysmal
depolarizing shift (PDS) remains the best cellular marker of an
epileptic event, corresponding to the interictal spike of the EEG. The
PDS represents a sustained movement of the neuronal resting potential
above threshold for 10--15 times the duration of a normal action
potential. Positive charge is initially carried in through Na+
channels, but Ca++ channels open within a few ms.
The Ca++ that enters the cell during the PDS is rapidly bound to
calcium binding proteins and sequestered in mitochondria. This
prevents the intracellular free Ca++ concentration from becoming
excessive and triggering a cascade of cytotoxic events. Below this
toxic threshold, Ca++ entry produces a proportional degree of K+
channel opening, which serves to terminate the PDS and induces a
prolonged after-hyperpolarization. Failure of this Ca++-dependent K+
current appears to be crucial to the transition from interictal spike
?Can epilepsy be cured?
Neurologists and neurosurgeons rarely speak of "cures" for migraine,
stroke, multiple sclerosis or epilepsy. More importantly, the focus is
The goal of epilepsy treatment is to stop seizures from occurring
without causing unacceptable side effects. In many patients this goal
can be achieved with drug therapy. For some people, drugs may be
needed only for a few years, whereas for others the need for medicine
may be lifelong.
What is the expert approach to epilepsy treatment?
Epilepsy is a complex disorder that requires the combined expertise of
a variety of specialists for the most effective care. Comprehensive,
quality care starting from the first doctor visit and continuing
through diagnosis, treatment, and follow-up necessitates a team
approach to the care of epilepsy patients. The Comprehensive Epilepsy
Program at Henry Ford Hospital focuses on multidisciplinary care,
bringing together a team of health professionals in a wide variety of
specialties to work together for the benefit of the patient.
?UCB today announced that the Committee for Medicinal Products for
Human Use (CHMP) of the European Medicines Agency (EMEA) has issued a
positive opinion to approve marketing authorisation of Keppra®
(levetiracetam) Concentrate (100 mg/mL) as an intravenous
administration and for use as adjunctive therapy in the treatment of
partial onset seizures with or without secondary generalisation in
adults and children of four years of age and older with epilepsy.?
?Keppra® is currently approved in the U.S. in tablet and oral solution
formulations for adjunctive therapy in the treatment of partial onset
seizures in adults and children four years of age and older with
epilepsy. n adults, the most common adverse events associated with
Keppra® in combination with other AEDs are somnolence, asthenia,
infection, and dizziness. Of these, most appeared to occur
predominantly during the first 4 weeks of treatment. In paediatric
patients the most common adverse events associated with Keppra® in
combination with other AEDs are somnolence, accidental injury,
hostility, nervousness, and asthenia. Please consult local prescribing
Keppra?s web site:
Wada Testing (also known as an intracarotid amytal test)
?This test was developed by Juhn Wada, a neurologist, in the 1940's.
The test was originally done to determine which side of the brain
controlled speech. Now it is used to test memory as well language
functions. It is a valuable tool in the evaluation of patients who are
being considered for epilepsy surgery, as it is the only known way to
examine the independent functions of the side of the brain that is
targeted for surgery.?
?When surgeons operate to relieve seizures, they need to know all
they can about the structure of the patient?s brain. The intracateroid
sodium ambobarbital, or Wada test, helps to identify the areas of a
person?s brain that control speech and memory functions. During this
pre-operative procedure, an angiogram of the brain is taken (an X-ray
of the brain?s blood vessels). A drug is then injected into the
patient that anesthetizes one side of the brain; the patient is asked
to respond to a series of memory and speech-related tests. From this
test, the neurosurgical team can determine where the areas of the
brain that control speech and memory are located, and thus avoid those
areas during surgery.?
?While most people with epilepsy achieve satisfactory control of
their seizures using medication alone, there still exists a large
number for whom medication does not achieve this goal. Some of these
people may benefit from a surgical procedure to improve control of
their epilepsy and enhance the quality of their lives. Epilepsy
surgery is not new. The first successful operation for epilepsy was
performed in 1886, and such treatment became common in the middle part
of the twentieth century. With tremendous advances in surgical
technique and diagnostic tools such as MRI and CT, the past two
decades have seen a significant increase in our ability to identify
people with epilepsy who might benefit substantially from surgical
treatment, as well as in the ease and safety of carrying out the
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Cerebral vascular disease + epilepsy
role of cerebral vascular disease + epilepsy
Cerebrovascular disease + epilepsy
small vessel vasculitis + epilepsy
etiology + epilepsy
Clarification of Answer by
22 Apr 2006 12:34 PDT
Helo again Sumers,
Thank you for your clarification.
Progressive small vessel disease, also known as small vessel
vasculitis, cause is often unknown.
The short answer: Small vessel vasculitis is caused by inflammation,
triggered by antibodies from an infection, vessel damage, or other
Here are the long answers!
?Few diseases in clinical medicine cause as much diagnostic and
therapeutic consternation as vasculitis (1-6). Vasculitis is simply
inflammation directed at blood vessels identified by histologic
examination. When blood vessel inflammation occurs, vessel wall
destruction with hemorrhage and aneurysm formation or stenosis due to
intimal hyperplasia can occur both of which may lead to tissue
ischemia and infarction. Vasculitis can be a primary process (no known
cause or association), or a phenomenon secondary to drug ingestion,
infection or the presence of a systemic disease (e.g., rheumatoid
arthritis) or to local factor such as trauma.?
?For instance, hepatitis C infection can found in association with
several different types of vasculitis such as polyarteritis nodosa
(PAN), cryoglobulenimic vasculitis (CV) and hypersensitivity/cutaneous
leukocytoclastic angiitis (CLA) (16). (Henceforth, leukocytoclastic
vasculitis (LCV) will be used to describe patients with the histologic
reaction pattern of small vessel neutrophilic vasculitis with or
without systemic disease.?
This site is discussing vasculitis in dogs, but the explanation is the
same, and easy to understand: ?Vasculitis is inflammation of the
vascular wall and can occur as a primary disease, but is more commonly
secondary to another concurrent disease (infections, neoplasia,
connective tissue diseases, drug reactions). Vasculitis may involve
only one organ system, such as the skin or may involve multiple organ
systems. Cutaneous vasculitis typically results from small vessel
vasculitis. Vasculitis is more often seen in dogs than in cats.
Etiology and pathogenesis
Vasculitis can be classified based on the histopathologic appearance:
lack or presence of inflammatory infiltrates as well as size and type
of vessels involved (small vessel vasculitis, arteritis, phlebitis).
However, these classifications do not reliably correlate with a
specific etiology. Most important is to differentiate between
vasculitis induced by infectious pathogens and vasculitis induced by
an immune-mediated process due to exogenous or endogenous antigens
(Table 1). In over 50% of cases an etiology cannot be determined.?
?Usually the cause of vasculitis is unknown. Sometimes it occurs as an
allergic reaction, for example, to medication. Vasculitis is not a
hereditary disease and it does not run in families. Some types may be
caused by infections, for instance with hepatitis viruses and some
bacteria?. There is an illustration on this page.
ANCA- anti-neutrophil cytoplasmic antibodies
Antibodies are molecules that are found in the blood stream. They are
mainly there to fight infections by targeting foreign molecules. ANCA
(anti-neutrophil cytoplasmic antibodies) stick to molecules found in
white blood cells rather than to foreign material. These antibodies
are often found in the blood of patients with small vessel vasculitis
(SVV). It is not clear whether ANCA cause vasculitis, but they can be
very helpful in the diagnosis. Unfortunately similar antibodies may be
found in other diseases, and not all vasculitis may be ANCA positive,
so the test is not 100% reliable as a way of making a diagnosis.
?Systemic vasculitides are characterized by aberrant immune responses
that result in inflammation and necrosis of blood vessels. The immune
dysfunction may be triggered by infection, autoimmune disease, or
exposure to a drug; often the cause is unknown. The categorization of
vasculitis by the size of the involved blood vessels is a useful guide
to diagnosis and therapy(1-3) (table 1). The most recent nomenclature
for vasculitis was developed by a multispecialty panel at the Chapel
Hill Consensus Conference on the Nomenclature of Systemic Vasculitis
(3). This article presents a review of the chief types of vasculitis
with an emphasis on important diagnostic features.?
? Schönlein-Henoch purpura
? Mixed cryoglobulinemia
Small to medium vessels
Small-vessel vasculitis typically involves the venules, capillaries,
and arterioles located in the skin, kidneys, and gastrointestinal
tract (6). The hallmark of the disease is palpable purpura, a slightly
raised, nonblanching eruption that usually begins in the lower
extremities (figure 2: not shown). Occasionally, the rash is vesicular
or slightly ulcerated. Skin biopsy reveals a leukocytoclastic
vasculitis characterized by disruption of the postcapillary venule
walls with nuclear debris from polymorphonuclear cells, fibrinoid
necrosis, and extravasation of red blood cells (figure 3: not shown).
An experienced clinician can identify palpable purpura on visual
examination, although skin biopsy may be useful when the diagnosis is
uncertain. Renal manifestations include proteinuria and renal
insufficiency. Colicky abdominal pain or melena may develop in
patients with gastrointestinal disease.?
?Most patients do not need treatment, because small-vessel vasculitis
is usually self-limited. Occasionally, however, the disease is
persistent or progressive. Patients should undergo serial examination
of the urine and the abdomen to detect development of renal or
intestinal involvement. Therapy with prednisone and other
immunosuppressant medications may be indicated in patients with
significant renal or gastrointestinal manifestations.?
?Vasculitis is inflammation of blood vessel walls. The clinical and
pathological features are variable and depend on the site and type of
blood vessels that are affected. Diseases in which vasculitis is a
primary process are called primary systemic vasculitides.?
?Small vessel vasculitides are being recognised more frequently,
mainly because of increased awareness. Estimates of incidence have
increased from fewer than 5 cases per million population in the early
1980s to over 20 per million. The early symptoms of these disorders
are non-specific with fever, malaise, arthralgia, myalgia, and weight
loss, and patients in whom such symptoms are persistent should be
screened for antineutrophil cytoplasmic antibodies (ANCA); have their
erythrocyte sedimentation rate and C reactive protein concentration
measured; and have their urine tested for blood with a dipstick. Early
diagnosis is essential to prevent potentially life threatening renal
and pulmonary injury. Delays in diagnosis are unfortunately common,
and this often leads to serious morbidity. Once respiratory or renal
disease develops, the course is usually rapidly progressive.?
?Secretory products of platelets serve important functions in
inflammation and thrombosis. Participation of platelets in the tissue
reaction associated with cutaneous small vessel vasculitis has not yet
been evaluated, so we systematically investigated the presence of
platelet aggregates in inflamed microvessels.
METHODS: Thirty-six biopsies containing vasculitis and 18 biopsies
with perivascular or interface type dermatitis were reviewed and
adjacent sections were immunohistochemically stained with anti-CD61
antibody recognizing GPIIbIIIa receptors on platelets and with
anti-von Willebrand factor (anti-vWF) antibody.
RESULTS: Platelet aggregates were observed in 27 (75%) of the
vasculitis biopsies and three (16.7%) of the perivascular dermatitis
biopsies, of which two (11%) had traumatic vessel damage. In all
vasculitis cases, platelet clumps were associated with diffuse
immunostaining of the perivascular stroma with the initiator of
platelet aggregation anti-vWF. In the non-vasculitis type of
dermatitis anti-vWF staining remained strictly limited to the
cytoplasm of endothelial cells in 10 cases, and in eight cases there
was also slight diffuse perivascular staining, albeit less extensively
than in vasculitis cases.
CONCLUSION: Formation of platelet aggregates appears to play a thus
far unrecognized role in cutaneous small vasculitis. Secretory
products of platelets will likely contribute to the inflammatory
response and tissue damage in vasculitis. Moreover, platelet
immunohistochemistry may be helpful in the diagnosis of microvascular
damage in paraffin sections.?
?The vasculitides are a heterogeneous group of diseases that are
characterized by the inflammation of blood vessels. The clinical
manifestations depend largely on the size and type of blood vessel
involved and the extent of vascular inflammation. A definitive
diagnosis of vasculitis usually requires angiography or a biopsy from
clinically involved tissue that shows typical histologic features.
Vasculitis can affect multiple organs.
Pulmonary vasculitides are classified according to the size of blood
vessels involved. Large-vessel pulmonary vasculitis includes giant
cell and Takayasu arteritis, although pulmonary involvement in these
entities is rare. Medium-sized blood vessel vasculitis is associated
with Behçet disease, typically, in the form of pulmonary artery
aneurysms. Small-vessel pulmonary vasculitis includes Wegener
granulomatosis (WG), Churg-Strauss syndrome, isolated pauci-immune
pulmonary capillaritis, and microscopic polyangiitis (MPA).?
?Henoch-Schönlein purpura (HSP) is an immunoglobulin (Ig) A-mediated
small-vessel vasculitis that predominantly affects children but also
is seen in adults. HSP is a subset of necrotizing vasculitis
characterized by fibrinoid destruction of blood vessels and
leukocytoclasis. Clinical manifestations primarily include palpable
purpura, arthralgia or arthritis, abdominal pain, gastrointestinal
(GI) bleeding, and nephritis. The most serious long-term complication
from HSP is progressive renal failure, which occurs in 1-2% of
?Mixed cryoglobulinemia (MC) is a chronic autoimmune disorder that is
almost always associated with chronic liver inflammation due to
hepatitis C virus (HCV) infection. It is a combination of an immune
complex disorder and a lymphoproliferative disorder, with distinct
symptoms caused by each aspect of the disease. MC is believed to
develop when chronic liver inflammation causes B cells to grow out of
control and produce excessive amounts of antibodies, especially
anti-IgG antibodies known as rheumatoid factors.?
?Cryoglobulinemia is a medical condition that is caused by proteins
called cryoglobulins present in the blood. Cryoglobulins are abnormal
proteins that by definition have the unusual property of precipitating
from the serum specimen in the laboratory when it is chilled and
redissolving into the serum upon rewarming.
Cryoglobulins may or may not be causing disease. Cryoglobulins can
accompany another condition (such as dermatomyositis, multiple
myeloma, or lymphoma) or be an isolated condition themselves, called
Treatment can be tricky, and would depend on which form of vasculitis
a patient has. Steroids and certain forms of chemtherapy have been
"Novel immunosuppressive approaches have been examined including
preliminary studies using biologic agents, such as antagonists of
tumor necrosis factor and monoclonal antibodies to B lymphocytes."
"Doctors often treat patients with ANCA-positive small-vessel
vasculitis with drugs that suppress the immune system. Most patients
get better with treatment, but some do not. Some also relapse during
or after treatment, meaning the disease and symptoms return and more
treatment is usually required.
Because of fear of relapse, doctors often give drugs to patients with
small-vessel vasculitis for a long time. Giving the patients drugs for
a longer time increases the risk for serious side effects. If doctors
could identify which patients were more likely to relapse, they could
treat them for a longer time and spare patients who are unlikely to
relapse from potential side effects from long-term treatments."
" Treatment of patients with microscopic polyangiitis and Wegener's
granulomatosis has three phases: (1) induction of remission, (2)
maintenance of remission, and (3) treatment of relapse.11 Current
induction therapy often consists of cyclophosphamide (Cytoxan) and
corticosteroids. For aggressive disease, use of high-dose intravenous
methylprednisolone for three days is recommended, combined with
intravenous or oral cyclophosphamide.11 Tapering doses of prednisone
should follow, along with cyclophosphamide maintenance for 12 to 18
months. The lowest dosage of steroids that controls the disease should
be used, and infection should be considered if the symptoms appear to
exacerbate. For patients in sustained remission at 12 months, the use
of all medications may be gradually discontinued. Patients whose
symptoms are under good control must, nevertheless, be closely
followed at six-month intervals for signs and symptoms of relapse.
During treatment with these agents, complete blood counts and liver
function tests should be performed periodically.11
Other treatment regimens that may be of benefit include methotrexate,
azathioprine (Imuran), trimethoprim-sulfamethoxazole (Bactrim,
Septra), plasma exchange, cyclosporine (Sandimmune), intravenous
immunoglobulin, and monoclonal antibodies. 11
During the use of potentially ulcerogenic immunosuppressive therapy,
patients may be given H2-blockers or proton-pump inhibitors.
Prophylactic treatment with fluconazole (Diflucan) orally for fungal
infection may be considered, as well as trimethoprim-sulfamethoxazole
(480 mg) three times weekly for prophylactic treatment of patients
with pneumocystis carinii prophylaxis."
In summary, something triggers inflammation and antibody production
that causes the small vessel vascuitis, be it in the brain, kidneys,
skin, or elsewhere.
I hope this has helped. Feel free to request another Answer
Clarification, before you rate, if anything is unclear.
Clarification of Answer by
27 Apr 2006 22:19 PDT
Hi again Sumers,
Perhaps sites below will better explain small vesel vasculitis,
which can occur in many areas of the body. My clarification answer did
refer to the brain in some of the sites. Did you check all the sites
completely? I'm not able to post but a small amount from each web
Small vessel vasculitis affects just that, small vessels and small
vessels are present in the brains! For some unknown reason, the body
"turns" against a vessel wall, producing inflammation, and causing
vasculitis. There are several forms of vasculitis, which are described
on sites int he original answer, as well as this one. Vasculitis of
the brain is not different than vasculitis in other parts of the body,
but the sequlae are. Brain vasculitis can cause strokes and seizures,
while vasculitis of the kidney can cause kidney failure.
"What causes CNS vasculitis?
The underlying cause of CNS vasculitis is poorly understood. However,
it is widely accepted that an autoimmune mechanism is responsible for
the disease. When the body detects foreign pathogens it directs
antibodies against them. These antibodies bind to the foreign
organisms (or antigens) and form antigen-antibody complexes. During
the development of CNS vasculitis these complexes are deposited in the
walls of certain blood vessels supplying the brain and spinal cord.
This deposition results in the release of chemicals which attract
defensive white blood cells to the vessel wall. These white cells
infiltrate the vessel wall, ingest the antigen-antibody complexes, and
release enzymes which damage the vessel wall. The end result is the
inflammation and death of the blood vessel, and the death secondarily
of the tissue that the vessel no longer supplies.
What symptoms are associated with CNS vasculitis?
The symptoms associated with CNS vasculitis are varied. However, many
patients with this disorder follow a similar clinical course. Patients
usually develop headaches as an initial symptom. This may be
accompanied by changes in the patients mental status. These symptoms
are then followed by weakness over parts of the body, seizures, ataxia
(an inability to smoothly coordinate muscle movements), or even coma.
In fact, it is not uncommon for a patient to exhibit the signs of a
"Other treatments to reduce the auto-immune attack on the blood
vessels of the brain may include plasmapheresis (removal of proteins
including antibodies from the bloodsteam), and immunosuppression with
drugs such as cyclophosphamide. Because the suppression of the immune
system can result in a number of side effects the patient's overall
medical status is carefully monitored by the intensive care unit."
"Vasculitis describes a large number of conditions. Vasculitis can
be primary (the vessel inflammation occurs spontaneously, with no
other associated disease process) or secondary (the vessel
inflammation occurs due to some other preexisting disease). Secondary
vasculitis can be a manifestation of a large number of disease
processes, including a variety of connective tissue or autoimmune
diseases such as rheumatoid arthritis, systemic lupus erythematosus,
Raynaud's phenomenon, Sjogren's syndrome, sclerodactyly, polymyositis,
and dermatomyositis, as well as sarcoidosis, malignancy, hepatitis B
and hepatitis C infections, allergic reactions to antibiotics and/or
diuretics, and severe bacterial infections such as endocarditis,
pneumonia, meningitis, gonorrhea, or syphilis."
"In vasculitis, the inflammation response has gone awry: it may be
kicked off initially by the presence of an invader such as vasculitis
secondary to a severe bacterial infection; it may be part of an
overall immune system over-reactiveness as occurs when vasculitis
occurs secondary to an autoimmune disease such as systemic lupus
erythematosus and rheumatoid arthritis; or it may erupt spontaneously
as in cases of primary vasculitis. The end results, however, are
inflammation and destruction of blood vessel walls, blood clot
blockages within blood vessels, aneurysms (weakened bulging areas of
blood vessel walls which can rupture, causing catastrophic bleeding),
and oxygen deprivation of the affected organs and/or tissues, leading
to damage and destruction of various tissue or organs throughout the
"Brain and nervous system. When vasculitis affects the nervous system,
a variety of symptoms may result. Vasculitis of blood vessels in the
brain can lead to headaches, confusion, personality changes, seizures,
and coma. Depending on the area of the brain affected, other senses
may suffer, including vision, hearing, and/or balance. Vasculitis of
the nerves that provide sensation to the arms or legs can lead to pain
and paresthesias (odd sensations of tingling, burning, pinpricks,
lightning-flashes of pain, or numbness). A stroke occurs when an area
of the brain tissue is completely deprived of oxygen, causing severe
damage or destruction. The results of a stroke may be temporary or
permanent, and the specific types of potential disability depend on
what functions are normally controlled by the area of brain injured by
"Medications that calm the immune system and decrease inflammation are
the mainstay of treatment for the various types of vasculitis. These
include nonsteroidal anti-inflammatory medications (such as ibuprofen
or aspirin) and corticosteroids (such as prednisone). More severe
cases of vasculitis may require potent immunosuppressant drugs (such
as cyclophosphamide or azathioprine)."
"Background: Leukocytoclastic vasculitis (LCV) is a histopathologic
term commonly used to denote a small-vessel vasculitis. Many possible
causes exist for this condition, but a cause is not found in as many
as 50% of patients.
The disorder may be localized to the skin, or it may manifest in other
organs. The internal organs most commonly affected are the
gastrointestinal tract and the kidneys. Joints are also commonly
affected. The prognosis is good when no internal involvement is
present. The disorder may be acute or chronic."
" * Antibiotics are the most common drugs that can cause cutaneous
vasculitis, particularly beta-lactams. Nonsteroidal anti-inflammatory
drugs and diuretics also frequently cause vasculitis. However, almost
all drugs are potential causes.
* Various infections may be associated with vasculitis. Upper
respiratory tract infections (particularly beta-hemolytic
streptococcal infection) and viral hepatitis, particularly hepatitis
C, are most often implicated. HIV infection may also be associated
with some cases of cutaneous vasculitis. Ascertaining whether a drug
(eg, antibiotic) or an infection (eg, upper respiratory infection) is
responsible for the disease is impossible because the occurrence of
vasculitis postdates infection and the drug used to treat the
* Foods or food additives may cause vasculitis."
"Vasculitis can appear suddenly in someone who has previously been
completely well ? doctors call this primary vasculitis. Vasculitis can
also appear in people who have an established disease such as
arthritis ? this is called secondary vasculitis. It may happen, for
example, with rheumatoid arthritis, systemic lupus erythematosus (SLE)
or Sjögren's syndrome (see arc booklets 'Rheumatoid Arthritis',
'Lupus', 'Sjögren's Syndrome')."
"Vasculitis in small arteries ? this includes Wegener's
granulomatosis, microscopic polyangiitis and Churg?Strauss syndrome.
Vasculitis in the small arteries can also be a consequence of
rheumatic diseases, including rheumatoid arthritis and systemic lupus
erythematosus. It can happen with infections including hepatitis and,
very occasionally, with different types of cancers, including
leukaemia and lymphomas."
"Vasculitis may occur as a primary process or may be secondary to
another underlying disease .
The exact mechanisms underlying these disorders are unclear. Three
different pathogenic models of disease have been advanced to help
explain why the lesions of a particular vasculitic syndrome are only
found in specific vessels :
* The distribution of the antigen responsible for the vasculitis
determines the pattern of vessel involvement.
* The recruitment and accumulation of the inflammatory infiltrate
is determined by the endothelial cell, including the expression of
adhesion molecules, the secretion of peptides and hormones, and the
specific interaction with inflammatory cells. Some endothelial cells
are therefore able to attract inflammatory cells, while others are
* Nonendothelial structures of the vessel wall are involved in
controlling the inflammatory process. In addition to the endothelial
cells which provide costimulatory function, other cellular components
serve as antigen-presenting cells and contribute proinflammatory
I trust this additional information will fully explain the situation.
Oh, as far as drugs, antibiotics are to blame in some cases. Those are
the only drugs implicated, that I could find.
I wish your daughter the best!