SYMPTOMS
or Manifestations of
CNS-SLE
In addition to headache, NP-lupus can cause other
neurological disorders, such as mild cognitive
dysfunction, organic brain syndrome, peripheral
neuropathies, sensory neuropathy, psychological problems
(including personality changes, paranoia, mania, and
schizophrenia), seizures, transverse myelitis, and
paralysis and stroke.
Characteristic abnormalities are frequently found on
brain MRI, lumbar puncture and electroencephalogram.
The diagnosis is not always straightforward.
Although there are no "definitive" tests for
CNS-lupus, there is a type of brain scan called a
"SPECT brain scan" that may be positive even
when an MRI brain scan is normal.
There is also a blood test called an
anti-ribosomal-P-antibody that, when it is positive, may
be helpful in establishing the diagnosis.
INDICATORS
for Diagnosis of CNS-SLE
NP-SLE is extremely variable.
The revised criteria for the diagnosis of NP-SLE lists
only coma and seizures, however, the clinical indications
of the disease can vary from profound and life
threatening neurological disturbances to ongoing mild
complaints such as headaches or behavioral disturbances.
Further confounding the ability to diagnose NP-SLE is the
fact that a patient may develop a psychiatric illness as
a consequence of having lupus without having NP-SLE.
Eg: 'reactive depression' or "Why
me?" and/or the patient may develop a
psychiatric illness entirely separate from the lupus
diagnosis entirely.
Also, certain therapies may produce psychiatric
disturbances.
Steroid therapy, particularly in high doses, can produce
psychotic behaviors.
As well, the fact that many patients with systemic lupus
present with cognitive function difficulties, makes it a
challenge for the physician to differentiate between what
is related to active systemic LUPUS, what is related to
corticosteroid treatment, and what may be related to
depression or neuropsychiatric lupus.
As the therapy used to treat patients with SLE can cause
psychiatric problems, it makes it very difficult for the
rheumatologist to decide if the patient is suffering from
primarily NP-SLE, reactive depression or the side effects
of steroid therapy.
It is usually necessary to rule out other conditions that
may mimic central nervous system manifestations of
systemic lupus erythematosus, including infection and
toxic metabolic states.
CNS-SLE DIAGNOSIS
The rheumatologist has a battery of diagnostic tests and
procedures which can aid him in making the diagnosis of
NP-SLE.
These include studies of spinal fluid, Brain Scan,
Electroencephalography (EEG), Cat Scan and psychometric
testing.
But at present, there is no single test or group of
procedures that can unerringly diagnose the illness in
all cases.
The tests can merely increase the index of suspicion that
NP-SLE is present.
To put this in the proper perspective, a patient who has
multi-organ involvement and signs of severe NP-SLE, such
as seizures, strokes, etc., can easily be given the
diagnosis of NP-SLE.
However, these patients represent the exception to the
rule.
The typical patient shows joint pain, skin rash, a
positive ANA and mild symptoms of NP-SLE such as
headaches and severe anxiety.
For such a patient, the rheumatologist is confronted with
the difficult decision of determining if the headaches or
anxiety are truly caused by NP-SLE.
Moreover, for certain patients, their only indications of
SLE may be NP-SLE, further adding to the diagnostic
quandry.
Multiple investigators are attempting to find better
tests to diagnose NP-SLE, such as the test for
antinerve cell antibodies.
TREATMENT of CNS-SLE
Since the indications of NP-SLE can vary from
very severe to mild symptoms, should all patients with
NP-SLE be treated with steroids, irrespective of the
intensity of the disease activity?
This is a controversial subject among rheumatologists.
Patients with central nervous system manifestations of
lupus erythematosus who present with organic brain
syndrome or coma can be treated with intravenous
methylprednisolone pulse therapy.
Patients with severe or resistant symptoms may also
require treatment with intravenous cyclophosphamide
and/or plasmapheresis.
Conclusively, individuals with major
manifestations of NP-SLE require aggressive
therapy with high dose steroids and perhaps
immunosuppressive drugs, plus other appropriate treatment
such as anti-seizure medication.
But what should be done for patients with minor
manifestations of neurologic and psychiatric
NP-SLE?
At present, the treatment of symptoms is most judicious,
such as the control of headaches and migraines with
appropriate medications.
CONCLUSIONS
The study of NP-SLE is still in its infancy, but
certain strides are being made.
For example, it appears that at least one subset of
patients with NP-SLE has been identified.
In these patients, NP-SLE is indicated by strokes,
apparently because an autoantibody predisposes the brain
to develop blood clots leading to strokes.
An enormous amount of investigative effort is required to
further understand the mechanisms relating to NP-SLE.
Hopefully a better understanding of the involved
mechanisms will lead to the development of more rational
therapy for the many manifestations
of CNS-SLE.
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