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