Rasmussen encephalitis a rare entity of neurodegenerative disorder found in a 6 year old girl during my residency pleriod.

1. Rasmussen Encephalitis
Dr. Santosh Atreya
Phase B Resident
Department of Radiology & Imaging
BSMMU

2. History and Etymology
• It was first
described by
American
neurologist Theod
ore Brown
Rasmussen (1910-
2002) in 1958

3. Introduction
• Rasmussen encephalitis (RE), also known
as chronic focal encephalitis is an extremely
rare chronic inflammatory neurodegenerative
disease of unknown origin.

4. History
• A 6 year-old girl, daughter of a rickshaw puller
presented to pediatric OPD with a history of
febrile illness with clonic movements of the
left hand and leg since 5 years, she was
developing normally till the age of 1 year
with no significant illness, gradually she
developed difficulty in walking. With these
complain she was taken to local doctor where
they were counselled to be better as time
goes by.

5. Contd…
• For the past one year, she was having mild
frontal headache. Her perinatal period and
developmental milestones had been normal.
On examination ,her hearing, vision & speech
were intact. Other systemic examination was
apparently normal.
• Routine blood and cerebrospinal fluid
investigations were within normal range.

6. Epidemiology
• Most cases (85% cases) occur in children
under the age of 10 years .

7. Clinical presentation
• Patients frequently have generalised status
epilepticus.
• The seizures are intractable despite
aggressive medical management .

8. • Apart from seizures, patient may have
hemiparesis and speech disturbances, as
well as hemianopia (pertaining to
unilateral cerebral involvement).
• Mental deterioration-in adolescence.

9. Radiographic features

10. • Radiographic features are
usually isolated to a single
hemisphere, however bilateral
Rasmussen encephalitis has
also been rarely described in
numerous case reports.
Radiological study is an important
tool for early diagnosis and
excluding differential diagnoses

11. CT SCAN
• CT may not show any
specific feature in early
imaging; however, patchy
hypo dense attenuation
areas may be seen.
• Late stage disease may
show unilateral cortical
atrophy

12. • MRI
• T1: unilateral cortical
atrophy with ex-vacuo
ventricular dilatation
• T2: hyperintense signal
areas in the affected
hemisphere
• DWI: restricted diffusion
may be seen in altered
signal areas
• T1 C+ (Gd): no significant
post-contrast enhancement

13. PET studies

14. Differentials
• Differential Diagnosis: Differential diagnostic
considerations usually need to cover:
• 1.Dyke-Davidoff-Masson syndrome
• 2.Sturge-Weber syndrome
• 3.hemi-megalencephaly.

15. Dyke-Davidoff-Masson syndrome
Cerebral hemiatrophy
characterised by :
Thickening of skull vault(compensatory)
Enlargement of PNS & mastoid air cells
Ipsilateral falcine displacement
Capillary malformations

18. Sturge-Weber syndrome
• Tram-track sign of
cortical and sub
cortical calcification,
with or without
ipsilateral prominence
of choroid plexus- hall
mark radiological
sign.

19. Hemi-megalencephaly.

20. Often Cortex appears
thickened “lumpy-bumpy” on
T1 scans
I/L ventricle is usually
enlarged and deformed
In severe cases almost no
normal hemispheric
architecture can be
discerned

21. T2WI –
• shows areas of patchy &
polymicrogyria with
indistinct borders between
gray & white matter
• White matter signal
intensity on T2/FLAIR is
often heterogenous with
cysts and gliosis-like
hyperintensity

22. Treatment and prognosis
• Treatment with high-dose methylprednisolone
and intravenous immunoglobulin has been
successful, further supporting the
autoimmune nature of the disease.
• Functional hemispherectomy is the only
definitive treatment in refractory cases, with
most patients having either no or less
frequent seizures .

23. Reference:
• Binoj Varghese,MK Aneesh, Navdeep Singh,and Percival Gilwaz. A Case of Rasmussen Encephalitis: The Differential
Diagnoses and Role of Diagnostic Imaging. Oman Med J. 2014 Jan; 29(1): 67–70.
• Rimzim Gupta, Rashi Bhargava, Rajesh Kumar Gupta, Prem Prakash Gupta Rasmussen encephalitis: A case report
Indian J Child Health Vol 2 | Issue 2 | Apr - Jun 2015
• Joseph Vimal, Ramesh Nagarajan, and Deepika Sriram Rasmussen’s encephalitis: A case report Australas Med J.
2015; 8(3): 80–84.
• Sadhanandham Shrinuvasan, Ranganathan Chidambaram Rasmussen's encephalitis: A rare case report CHRISMED
J Health Res 16-Mar-2015
• Lei Chen, M.D. Peimin Feng, M.D. Dong Zhou, M.D. Case Reports of Rasmussen’s Syndrome and Literature Review
J Neuropsychiatry Clin Neurosci 24:3, Summer 2012
• Yvonne Hart Radcliffe Infirmary, Oxford, UK Rasmussen’s encephalitis Epileptic Disorders Vol. 6, No. 3, September
2004
• C. G. Bien,1 T. Granata,2 C. Antozzi,2 J. H. Cross,3 O. Dulac,4 M. Kurthen,1 H. Lassmann,5 R. Mantegazza,2 J.-G.
Villemure,6 R. Spreafico2 and C. E. Elger Pathogenesis, diagnosis and treatment of Rasmussen encephalitis A
European consensus statement Brain (2005), 128, 454–471
• Deepak Jain,1 Hari Krishan Aggarwal,1 Shivraj Goyal,1 and Ansul Mittal1 Dyke–Davidoff–Masson syndrome: A rare
case report ran J Neurol. 2014 Oct 6; 13(4): 255–256
• Siddarth Ragupathi1, Ajit Kumar Reddy2, Annitha Elavarasi Jayamohan2,Prakash Manikka Lakshmanan3 Sturge-
Weber syndrome: CT and MRI illustrations BMJ Case Reports 2014; doi:10.1136/bcr-2014-205743
• Greg James & Mano Shanmuganathan & William Harkness Hemimegalencephaly without epilepsy: case report
Childs Nerv Syst Springer-Verlag Berlin Heidelberg 2014