Transcript Document 7315313

Case presentation
Dr Aysha Alshareef
Neurology consultant, Assistant
professor
history
the case was referred to neurology team from ob
ward
she was 34 y old chadian F, P1+0 ,2 days post CS
Acute Confusion ,recurent generalized GTC
seizure
?headache, h/o other neurological symptoms ?
No fever.
No similar attak in the past
Drugs: unremarkable
Social:married ,living in Jeddah
No h/o hypertension, or other medical illness
O/E
Vital sign :BP 189/88, afebrile
General : no lower limb edema
Neurological:
no neck stifness
She was disoriented ,no papilledeoma
No focal neurological signs,moving all
limbs,hyper reflexea,planter were
bilaterally down going
Other systems :unremarkable.
Differential diagnosis
Post partum +Recurrent seizure
+encephalopathy
Eclampsia
Hypertensive encephalopathy
Cerebral venous thrombosis
Arterial stroke
Others :metabolic , encephalities…
Work up
CBC
U&E
LFT
Urine for protien: -ve
MRI
P
R
E
S
: posterior
: Reversible
: encephalopathy
: syndrome
RPES
is a clinical radiologic syndrome of
heterogeneous etiologies that are
grouped together because of
similar findings on neuroimaging
studies.
Posterior reversible
leukoencephalopathy syndrome
It is also often referred to as:
Reversible posterior cerebral edema
syndrome
RPLS (reversible posterior
leukoencephalopathy syndrome)
Hyperperfusion encephalopathy
Brain capillary leak syndrome
it was first codified as a single named syndrome in
a 1996 .
This described a clinical syndrome of insidious
onset of headache, confusion or decreased level
of consciousness, visual changes, and seizures,
which was associated with characteristic
neuroimaging findings of posterior cerebral white
matter oedema.
N Engl J Med 1996 Feb 22;334(8):494-500.
EPIDEMIOLOGY
(RPES) is increasingly recognized and reported
in case reports and case series
however, the incidence of RPES is not known.
Patients in all age groups appear susceptible
AJNR Am J Neuroradiol 2002 Jun-Jul;23(6):1038-48.
reported cases exist in patients as young as two
years and as old as 90 years.
Case series suggest that PRES is more common
in women, even when patients with eclampsia
are excluded .
Neurology 1998 Nov;51(5):1369-76
PATHOGENESIS
The pathogenesis of PRES
remains unclear, but it appears to
be related to:
disordered cerebral autoregulation
and
endothelial dysfunction.
Autoregulatory failure
Endothelial dysfunction
vasodilatation
capillar leakage
hyperperfusion
disruption BBB
Vasogenic edeoma
Anatomic distribution :
WHY WHITE MATTER DISEASE?
The cortex, structurally more tightly packed than the white
matter, resists accumulation of edema, hence predilection of
abnormalities to be seen in the white matter
WHY POSTERIOR REGION ?
A histochemical study revealed a greater concentration of
adrenergic nerves around pial and intracerebral vessels in
the anterior circulation than posteriorly . This observation
may explain why the hyperperfusion and edema is mainly
seen in the posterior circulation in RPLS.
Acta Physiol Scand 1981
Feb;111(2):193-9
Clinical presentation
The clinical syndrome of reversible posterior
leukoencephalopathy syndrome (RPLS) is characterized by:
Headaches
Altered consciousness
Visual disturbances
Seizures
The headache is typically constant, nonlocalized, moderate
to severe, and unresponsive to analgesia .
Altered consciousness ranges from mild somnolence to
confusion and agitation, progressing to stupor or coma in
extreme cases .
Seizures are usually generalized tonic clonic; they may begin
focally and often recur. Status epilepticus has been reported
Preceding visual loss or visual hallucinations suggest
occipital lobe origin in some patients.
Intern Med J 2005 Feb;35(2):83-90
Signs
Visual perception abnormalities are often detectable.
Hemianopia, visual neglect, auras, visual hallucinations, and
cortical blindness may occur . The latter may be
accompanied by denial of blindness (Anton's syndrome).
The funduscopic examination is often normal, particularly in
eclamptic and chronically hypertensive patients, but
papilledema may be present with accompanying flameshaped retinal hemorrhages and exudates.
The deep tendon reflexes are frequently brisk with Babinski
signs often present .
. Other focal neurologic deficits are rare.
Hypertension is frequent but not invariable. The hypertensive
crisis may precede the neurologic syndrome by 24 hours or
longer .
Intern Med J 2005 Feb;35(2):83-90
Risk factors
Common:
Hypertension encephalopathy
Eclampsia
Acute and chronic renal failure
Immunosuppressive agents and
cytotoxic drugs
Acta Physiol Scand 1981 Feb;111(2):193-9
Immunosuppressive and
immunomodulatory drugs
Cyclosporine A ,
Bevacizumab,
Cisplatin Combination chemotherapy, Cytarabine Gemcitabine
Interferon-alpha
Intravenous immunoglobulin
Methotrexate
Rituximab
Sirolimus
Sorafenib
Sunitinib
Tacrolimus
Vincristine
Risk factors
Other reported causes:
Hemolytic and uremic syndrome
Collagen vascular disorders
leukemia
Behcets syndrome
TTP
HIV
Acute intermittent prophyria
Hypercalcemia,hypomagnesmia
Contrast media exposure
Cryoglobulinemia
Hypertensive
encephalopathy
• sever hypertension, Rapidly developing, or
intermittent hypertension carries a particular risk
for hypertensive encephalopathy .
•untreated or under treated chronic hypertension
also carry risk of PRES
• PRES is more common, in patients with
comorbid conditions
•
Eclampsia
Some suggest that PRES (typical clinical
syndrome and neuroimaging findings)
could be considered an indicator of
eclampsia, even when the other features of
eclampsia (proteinuria, hypertension) are
not present .
Br J Obstet Gynaecol 1997 Oct;104(10):1165-72.
Immunosuppressive therapy:
The neurotoxic effects of these therapies are well known but
still poorly understood.
Toxic levels of medications are not required for the
development of PRES
prior exposure to the drug does not appear to be protective
.
Even after several months of exposure to the drug, patients
with therapeutic levels can be symptomatic .
Mol Interv 2004 Apr;4(2):97-107.
Cyclosporine is one of the more common
cytotoxic therapies associated with PRES.
After renal toxicity, neurotoxicity is the most
serious side effect with cyclosporine.
affecting 25 percent to 59 percent of transplant
patients.
Hypomagnesemia, and hypertension have all
been implicated in facilitating cyclosporine
neurotoxicity .
J Biol Chem 2002 Aug
16;277(33):29669-73. Epub 2002 Jun 5.
DIFFERENTIAL DIAGNOSIS :
Arterial stroke , Particularly in cases with a sudden onset of neurologic symptoms, the
presentation can mimic bilateral posterior cerebral artery infarctions ("top of the basilar
syndrome").
cerebral venous thrombosis
Others :
demyelinating toxic or metabolic encephalopathy, , vasculitis, or encephalitis , ,among
others .
It is important to distinguish between
PRES and ischemic stroke, as the
treatment of hypertension may be
very different in these conditions.
J Neurol Neurosurg Psychiatry 2000 Aug;69(2):248-5
NEUROIMAGING:
Neuroimaging is essential to the diagnosis of reversible posterior
leukoencephalopathy syndrome (PRES)
magnetic resonance imaging (MRI) is the best modalities .
Typical findings are symmetrical white
matter edema in the posterior cerebral
hemispheres, particularly the parietooccipital regions, but variations do occur .
Complete resolution of neuroimaging findings within days to
weeks is expected.
.
J Neuroimaging 2004 Apr;14(2):89-96
DIAGNOSIS :
There are no specific diagnostic
criteria for reversible posterior
leukoencephalopathy syndrome
(RPLS).
clinical and radiological findings.
PREVENTION AND TREATMENT
(PRES) should be promptly recognized,
since it is usually reversible.
Treating clinicians should have a high
clinical suspicion in the appropriate settings
Treat underlying risk factors(
hypertension,eclampsia, stop
immunosupression )
Hypertension
with lowering blood pressure , patients will often improve
dramatically.
For patients with lower levels hypertension, lowering blood
pressure is also recommended to treat PRES
this goal should be achieved within two to six hours, with the
maximum initial fall in BP not exceeding 25 percent of the
presenting value.
Lancet 2000 Jul 29;356(9227):411-7
IV drugs
such as
nicardipine, labetalol, and nitroprusside are
effective and safe in reducing the blood
pressure to a desirable range] .
Oral antihypertensive are not usually
effective to treat PRESS.
PROGNOSIS:
Most case series and case reports
suggest that (PRESS) is often
benign.
In many cases,PRES seems to be
fully reversible within a period of
days to weeks, after removal of
the inciting factor and control of
the blood pressure.
However, one of the largest case series
reported highlights the potential grave
consequences of this disorder; among 22
patients studied, six died and many
survivors had permanent neurologic
disability
. Death may result from progressive
cerebral edema, intracerebral hemorrhage,
or as a complication of the underlying
condition .
Arch Neurol. 2008 Feb;65(2):205-10
SUMMARY AND
RECOMMENDATIONS
(PRES) is a neurologic syndrome defined by clinical and
radiologic features.
The typical clinical syndrome includes headache, confusion,
visual symptoms, and seizures. Typical MRI findings are
consistent with vasogenic edema and are predominantly
localized to the posterior cerebral hemispheres. DWI can be
helpful in distinguishing PRES from stroke.
Prompt reduction of blood pressure or withdrawal of
immunosuppressive agents leads rapid reversal of the
syndrome
It is important to distinguish between PRES and ischemic
stroke, as the treatment of hypertension may be very
different in these conditions.,