Aetiology
|
Basilar artery occlusion
(due to cardioembolism, trauma, hypercoagulable disorders, unknown)
Vertebral artery dissection
(+/- after neck traumatism)
Brainstem ischemic syndrome in NF2
Unknown
|
Diffuse high-grade glioma
(+/- 75%)
Focal low-grade glioma
Exophytic glioma
(low grade)
Langherans cell histiocytosis
Epidermoid/dermoid tumor
|
Listeria, Enterovirus, HSV
|
ADEM †
Multiple sclerosis
ANE ‡
(after influenza A, B, parainfluenza, HHV6)
Bickerstaff encephalitis
(ganglioside GQ1b antibodies, after Campylobacter jejuni or
Mycoplasma pneumoniae)
|
Mitochondrial disease
|
Central pontine myelinolysis
|
Specific symptoms
In addition of headaches, cranial nerve palsy, motor deficit
|
-
|
Ataxia
|
Fever
Encephalopathy
Seizures
Ataxia
|
+/- after a viral infection
Encephalopathy
Seizures
Bickerstaff :
Ophtalmoplegia, ataxia, coma, areflexia
|
+/- decompensation after a viral infection
Multisystemic symptoms,
ataxia, dystonia, regression
|
severe alteration of plasma osmolality or hyponatremia
Encephalopathy
Confusion
|
Brain MRI
And other tests
|
T2 and flair hypersignal
pons > medulla
reduction in diffusion
|
T2 hypersignal
High grade glioma :
mass effect, oedema, infiltration, absent or
inhomogeneous contrast enhancement
Low grade glioma
focal, less oedema
Exophytic glioma
from the 4th ventricle, as low grade glioma
Langherans cell histiocytosis
With hypothalamic-pituitary lesions
(Epi)dermoid tumor
Focal, no oedema, reduction in diffusion
|
T2 hypersignal
multiple lesions, patchy, asymmetric, +/- abscess , +/-
supra-tentorial lesions
CSF § :
WBC ↑, protein normal or ↑, glucose : normal or ↓
Culture and PCR
|
T2 hypersignal
with supra tentorial lesions in white matter (except Bickerstaff
: only brainstem)
CSF § :
WBC ↑, protein ↑, normal glucose, +/-oligoclonal bands, ganglioside GQ1b
antibodies + in Bickerstaff
|
T2 hypersignal
Grey matter :
substantia nigra, medullary and pontine tegmentum, basal ganglia and
diffuse supra -tentorial leukoencephalopathy
Blood test :
lactate ↑, alanine ↑, urine organic acid : abnormal
CSF § : protein normal or ↑, lactate ↑
Molecular analysis
|
T2 hypersignal
Central pontine myelinolysis
central pons, +/- trident shape +/- midbrain and middle cerebellar
peduncles
Blood test :
Osmolality alteration
|
Treatment
|
Anticoagulation or Aspirin
+/- thrombolysis
|
According histology
|
Antimicrobial treatment
|
Corticoids
|
Vitamins and supportive therapy
|
Prevention
Supportive therapy
|
Outcome |
good except if size > 50% or
coma at presentation |
According histology |
Poor outcome in 50% |
According aetiology |
Poor |
Variable |