acute hemorragic leucoencehalitis dr.silambarasi stanley medical college
TRANSCRIPT
ACUTE HEMORRAGIC LEUCOENCEHALITIS
DR.SILAMBARASI
STANLEY MEDICAL COLLEGE
ABSTRACT
Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare form of ADEM's presentation due to acute brain vasculitis. Both will have same prsentation but ADEM requires immediate and aggressive treatment because this clinical scenario shows high mortality
Herein, we report a case of AHEM with remarkable abnormalities of brain magnetic resonance imaging. other than supportive management high dose of corticosteroid may save the life , our case is the case of Japanese Encephalitis presented with foci of demyelination associated with hemorrhagic areas and show significant improvement after high dose of cotricosteroid
CASE REPORT
previously healthy 1 Year Old Male Child admitted with Fever for 3
days .Fever was high grade No History Suggestive Of Focal
Sepsis/ acute diarreoeal disease / RTI / recent vaccination .
Developmental history child was normal, He is full term normally
delivered child who has cried immediately after birth… NO H/O
any previous hospital admission . No h/o any seizure disorder or
febrile disorder. On Admission, Clinical examination was
unremarkable except for, inconsolable cry and irritability. pincer
grasp obtained. Child was immunised upto date.. Kernig
sign ,brudzinski’s sign negative.Deep Tendon Reflexes normal
INVESTIGATIONS
Routine blood examination shows HB-12.8mg%, RBC -3.8lakhs/cumm WBC-
13,000,ESR-34meq/l. On Day 3 of admission child developed seizure and
treated with 15ug/kg of phenobarbitone. Clinical Diagnosis of
Meningoencephalitis was made out.
CSF tapping for Analysis was done before starting Empirical
antibiotics .CSF ANALYSIS reveals clear fluid with no web on
standing ,Cells275 WBCs/mL,( increased) with 83% segmented neutrophils
(Neutrophilic pleocytosis)Total protein -260 mg/dL, (increased) Glucose -79
mg/dL. Japanese B Encephalitis virus was isolated .child was further
evaluated as there was no significant clinical mprovement to the above
treatment
CT
T1 MRI
RL
Hypointensity in fronto parieto occipital cortex WM, splenium of corpus callosum
T2
Hyperintensity in fronto parieto occipital cortex WM,splenium of corpus callosum
FLAIR
Non suppressibe Hyperintensity in fronto parieto occipital cortex WM,splenium of corpus callosum
DWI/ADC
Areas of partial diffusion restriction in fronto parieto occipital WM
DWI/ADC
Areas of partial diffusion restriction in fronto parieto occipital WM,Splenium of CC
SWI mIP
Areas of blooming in fronto parieto occipital WM
No specific feature
DIAGNOSIS
Diagnosis of ACUTE HEMORRHAGIC LEUCOENCEPHALITIS made out .patient was treated with high dose of corticosteroid IV administration of high-dose intravenous methylprednisolone (30 mg/kg/day) for 5 days, followed by oral prednisolone (2 mg/kg/day) taper for 6 weeks.
…follow up images taken shows areas of improvement
SWI
T2
Post treatment follow up
DISCUSSION
AHLE represents the rare, severe extreme of a spectrum of CNS demyelinating
diseases, which includes the more common paediatric condition acute disseminated
encephalomyelitis (ADEM) Incidence of ADEM 8 per 10,00,000 2%of ADEM Present
as AHLE .Both are commonly preceded by upper respiratory infections or by HSV,
EBV, rubella, measles, mumps, or influenza virus, vaccination rarely Japanese
encephalitis, malaria, dengue infection, SCA. AHLE may more commonlyoccur in
young adults, contrary to ADEM, which more commonly diagnosed in children.
There is no gender predilection. Main etiology behind AHLE is the immune response
to preceding viral infection/vaccination. That may be Cell mediated or Humoral .
PATHOGENESIS
PATHOGENESIS:- PERIVENULAR DEMYELINATION
ASSOCCCIATED WITH HEMORRHAGE IS THE HALL MARK.
Complement Activation by AG-AB complexes - to the myelin
antigen leads to necrotizing venulitis with perivascular
hemorrhages . hemorrhagic lesions of the white matter, primarily
limited to cerebral hemisphere; pathological examination reveals
perivascular polymorphonuclear infiltrates; demyelination.
IMAGING
imaging demonstrate white matter involvement, rarely gray matter can also be
involved CT demonstrate regions of hypodensity within the white matter and
may demonstrate enhancement.On MR imaging, asymmetric unilateral or
bilateral white matter abnormalities of the cerebral hemispheres, particularly
the frontal and parietal lobes, which extend from the periventricular region to
the gray–white junction with relative sparing of the cortex Deep gray matter
involvement might be present. Low diffusivity is noted in most areas . It is
suggested that this diffusion abnormality may be caused by cytotoxic edema
secondary to the toxic effect of acute inflammation or acute vasculitis with
subsequent vessel occlusion, which might also explain vessel wall necrosis
causing haemorrhage .Contrast enhancement of the lesions is variable,
depending on the different age and severity in degree of the lesions because
the breakdown of the blood–brain barrier in the acute inflammatory phase is
the pathologic correlate of this appearance
MANAGEMENT
Supportive measures, empirical ICP monitoring and death
is the usual outcome. Studies have shown that Intensive
care, use of high-dose corticosteroid therapy,
immunoglobulins, cyclophosphamide, plasma
exchange(AUTOIMMUNE),dehydrating agents, and
surgical decompression(ICP) has led to survival in
some.SO To Conclude,AHLE is usually fatal, whereas full
recovery is the rule for patients with ADEM
ACKNOWLEDGEMENT –PROFESSOR,ASSOCIATE PROFESSOR, ASISSTANT PROFESSOR –STANLEY MEDICAL COLLEGE
References
. Tenembaum S, Chitnis T, Ness J, Hahn JS. International Pediatric MS Study Group. Acute disseminated encephalomyelitis. Neurology. 2007;68(16 Suppl 2):S23–36. [PubMed]
2. Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain.2000;123:2407–22. [PubMed]
3. Mikaeloff Y, Suissa S, Vallée L, Lubetzki C, Ponsot G, Confavreux C, et al. First episode of acute CNS inflammatory demyelination in childhood: Prognostic factors for multiple sclerosis and disability. J Pediatr.2004;144:246–52. [PubMed]
4. Leake JA, Billman GF, Nespeca MP, Duthie SE, Dory CE, Meltzer HS, et al. Pediatric acute hemorrhagic leukoencephalitis: Report of a surviving patient and review. Clin Infect Dis. 2002;34:699–703. [PubMed]
5. Rosman NP, Gottlieb SM, Bernstein CA. Acute hemorrhagic leukoencephalitis: Recovery and reversal of magnetic resonance imaging findings in a child. J Child Neurol. 1997;12:448–54. [PubMed]
6. Anlar B, Basaran C, Kose G, Guven A, Haspolat S, Yakut A, et al. Acute disseminated encephalomyelitis in children: Outcome and prognosis. Neuropediatrics. 2003;34:194–9. [PubMed]
7. Hurst EW. Acute hemorrhagic leukoencephalitis: A previously undefined entity. Med J Aust. 1941;1:1–6.
8. Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: A long-term follow