Case presentation

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• Case presentation
• Dr Aysha Alshareef
• Neurology consultant, Assistant professor

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history

the case was referred to neurology team from ob
ward she was 34 y old chadian F, P10 ,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 Socialmarried ,living
in Jeddah No h/o hypertension, or other medical
illness
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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.

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Differential diagnosis

• Post partum Recurrent seizure encephalopathy
• Eclampsia
• Hypertensive encephalopathy
• Cerebral venous thrombosis
• Arterial stroke
• Others metabolic , encephalities

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Work up

• CBC
• UE
• LFT
• Urine for protien -ve

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CT brain
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• MRI

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P R E S
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• P posterior
• R Reversible
• E encephalopathy
• S syndrome

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RPES

• is a clinical radiologic syndrome of
  heterogeneous etiologies that are grouped
  together because of similar findings on
  neuroimaging studies.

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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

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• 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 22334(8)494-500.
  
  

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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-Jul23(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 Nov51(5)1369-76
  
  

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PATHOGENESIS

•   The pathogenesis of PRES remains unclear, but
  it appears to be related to
• disordered cerebral autoregulation and
• endothelial dysfunction.

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• Autoregulatory failure
  Endothelial dysfunction
• vasodilatation
  capillar leakage
  
• hyperperfusion
  disruption BBB
• Vasogenic edeoma

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• 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
  Feb111(2)193-9

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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 Feb35(2)83-90

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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 flame-shaped 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 Feb35(2)83-90

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Risk factors

• Common
• Hypertension encephalopathy
• Eclampsia
• Acute and chronic renal failure
• Immunosuppressive agents and cytotoxic drugs
• 
  
  
• 
  
  Acta Physiol Scand 1981 Feb111(2)193-9
  

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Immunosuppressive and immunomodulatory drugs

• Cyclosporine A ,
• Bevacizumab,
• Cisplatin Combination chemotherapy, Cytarabine
  Gemcitabine
• Interferon-alpha
• Intravenous immunoglobulin
• Methotrexate
• Rituximab
• Sirolimus
• Sorafenib
• Sunitinib
• Tacrolimus
• Vincristine

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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

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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

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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
  Oct104(10)1165-72.

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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 Apr4(2)97-107.

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• 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 16277(33)29669-73.
  Epub 2002 Jun 5.

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• 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 Aug69(2)248-5

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• 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 parieto-occipital regions, but
  variations do occur .
• Complete resolution of neuroimaging findings
  within days to weeks is expected.
• 
  
  J Neuroimaging 2004
  Apr14(2)89-96.

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• DIAGNOSIS 
•  There are no specific diagnostic criteria for
  reversible posterior leukoencephalopathy syndrome
  (RPLS).
• clinical and radiological findings.

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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 )

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• 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 29356(9227)411-7

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• 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.

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• 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.

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• 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
  Feb65(2)205-10

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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.,

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thanks