Download Pediatric spinal nerve root enhancement

eEdE-200-6875
Pediatric spinal nerve root enhancement:
Clinical and differential considerations
Marinos Kontzialis1, Hans Michell2,
Andrea Poretti2, Thierry A.G.M. Huisman2
1Division
of Neuroradiology and 2Pediatric Radiology, The Johns
Hopkins University School of Medicine, Baltimore, MD, USA
ASNR 53rd Annual Meeting, Chicago, April 25-30, 2015
[email protected]
Disclosure
We have nothing to disclose
No relevant financial relations interfering
with our presentation
Purpose
Case-based review of pediatric nerve root
enhancement
Clinical and differential considerations
Introduction
Pediatric spinal nerve root enhancement:
Indicates blood-nerve-barrier disruption
Is a nonspecific finding
Case 1
2-year-old with ascending weakness
A
B
C
Diffuse nerve root enhancement in the cervical (A),
thoracic (B) and lumbar spine (C)
Guillain-Barré syndrome (GBS)
 Autoantibody-mediated acute motor weakness:
 Pathogenesis = molecular mimicry and cross reactive
immune reaction
 Prevalence: 1.2-2.3/100,000
 Usually preceding upper respiratory or GI infection
(Campylobacter jejuni)
 Maximum weakness within 4 weeks
 Progressive weakness > 8 weeks  chronic
inflammatory demyelinating polyneuropathy (CIDP)
 CSF = albumin-cytological dissociation
Guillain-Barré syndrome: MRI
1. Nerve root enhancement: > 90%
2. Different patterns of enhancement
Diffuse = 68%
Ventral roots = 27%
Dorsal roots = 5%
Guillain-Barré syndrome: MRI
Cranial nerve (CN) involvement = 50%
Bilateral CN V enhancement (arrows)
Differential diagnosis 1
Transverse myelitis:
Quicker presentation compared to GBS (hoursfew days vs. several days)
Sensory-motor level (not present in GBS)
MRI: spinal cord signal abnormalities +
enhancement
10-year-old with transverse
myelitis presenting with
quadriplegia over 24-48 h
Differential diagnosis 2
Guillain-Barré syndrome
West-Nile virus radiculitis
• Poliomyelitis-like syndrome with acute
flaccid paralysis + sensory sparing
• MRI: most often normal, but ventral
nerve roots enhancement is possible
• Can look identical to GBS
Case 2
 8-year-old with congenital HIV infection
A
B
C
Bilateral CN III enhancement (A) and diffuse nerve
root enhancement and mild thickening (B, C)
Chronic inflammatory
demyelinating polyneuropathy (CIDP)
Inflammation mediated demyelination
Prevalence: 1-7.7/100,000
Clinically heterogeneous, grossly symmetric
sensory and/or motor neuropathy
Develops over > 8 weeks (GBS < 4 weeks)
No preceding infection
CSF = increased protein, normal cell count
 supportive of nerve root inflammation
Chronic inflammatory
demyelinating polyneuropathy
CIDP may occur in the context of:
Hepatitis C
Inflammatory bowel disease
Monoclonal gammopathy of undetermined
significance (MGUS)
HIV/AIDS
Organ transplant
Connective tissue disorders
Chronic inflammatory
demyelinating polyneuropathy
MRI: enhancement + hypertrophy of root +
plexus possible  widespread inflammation
Can mimic neurofibromatosis type 1
16-year-old with thickening and mild enhancement of lumbar
plexus (arrowheads) and sacral nerve roots (arrows)
Case 3
 9-month-old from El Salvador with a 2week Hx of fever, lethargy, and emesis
A
B
C
D
Diffuse meningeal enhancement in the posterior fossa (B), around the cord (B),
and cauda equina nerve roots (D). Minimal clumping of the nerve roots (D).
Case 3
A
B
Basilar meningitis
C
CN V enhancement
CN VI, VII, VIII
enhancement +
rim enhancing lesion in
the right middle
cerebellar peduncle
Tuberculosis
Most common infectious cause of spinal
arachnoiditis (= inflammation of the
meninges)
CSF: increased protein, decreased glucose,
increased cells (mainly lymphocytes)
Nerve root enhancement = 30%,
predominantly smooth
Case 4
 3-year-old with subacute onset of bilateral facial
weakness and dysarthria
A
B
C
Diffuse nerve root enhancement in the cervical (A),
thoracic (B) and lumbar spine (C)
Case 4
CN III enhancement
CN V enhancement
CN VII + VIII
enhancement
Bannwarth syndrome
Lyme meningo-radiculo-neuritis caused by
spirochete Borrelia burgdorferi
Most common tick-borne disease in the US
Geography, recreational habits, season
(peak in the summer) are clues!
Confirmed by serum + CSF antibodies
CSF = lymphocytic meningitis
Erythema migrans in 89% of children =
facilitates the diagnosis
Bannwarth syndrome
MRI:
MS-like periventricular white matter lesions
Cranial nerve enhancement
Nerve root enhancement
In the appropriate geographic + seasonal
setting  facial diplegia/palsy = highly
suggestive of Lyme disease, especially
when coupled with erythema migrans
Case 5
 13-year-old with progressive polyneuropathy
Thickening of the cauda equina nerve roots
without evidence of enhancement (not shown)
Case 5
CN III thickening +
enhancement
CN V thickening +
enhancement
CN VII, VIII thickening +
enhancement
Charcot-Marie-Tooth disease
Hereditary motor + sensory neuropathies
Symmetric + predominately distal motor +
sensory disturbances, slowly progressive
course
MRI: typically associated with marked
thickening of the nerves (hypertrophic
neuropathies)
Differential diagnoses
 Metachromatic leukodystrophy (MLD) + Krabbe disease
 Can present with diffuse cranial nerve and cauda
equina enhancement
 May be simultaneous or precede typical white matter
abnormalities
 Consider measuring arylsulfatase A
+ galactocerebrosidase in all children
with unexplained cranial nerve and
caudal nerve root enhancement
MLD
Krabbe
disease
Case 6
 3-year-old with developmental delay + failure to thrive
Diffuse leptomeningeal +
subarachnoid enhancement
Diffuse thickening
of the nerve roots
Case 6
Avidly enhancing pineal mass with
diffuse leptomeningeal enhancement in
the posterior fossa + around the cord
Leptomeningeal enhancement
coating bilateral CN V
Pineoblastoma with
leptomeningeal carcinomatosis
Neoplastic causes of nerve root enhancement
in the pediatric population:
Medulloblastoma
Germinoma
Pineoblastoma
PNET
Ependymoma
Astrocytoma
Lymphoma
Leukemia
Smooth nerve root enhancement
Most common, but least specific
GBS
West Nile
virus
radiculitis
CIDP
Lyme disease Tuberculosis
Nerve root enhancement + thickening
CIDP
CMT disease
Leptomeningeal
carcinomatosis
(enhancement can
be nodular)
Summarizing table
Diagnosis
Clinical
CSF
Clues
Associations
MRI
GBS
<4 weeks
 protein
Ascending paralysis,
preceding infection
50% CN
involvement
nerve root enhancement
CIDP
>8weeks
 protein
Progressive
neuropathy
Underlying
disorder?
Nerve root enhancement +
thickening
Meningitis
Encephalopathy,
meningeal signs
 Protein
 cells
Fever
Empiric treatment
+ CSF
± leptomeningeal and
nerve root enhancement
TB
Encephalopathy,
meningeal signs
 Protein
 Cells
 Gluc
Think about risk
factors of exposure
Pulmonary TB
Meningitis/arachnoiditis,
tuberculomas, nerve root
enhancement
Lyme
Rash
CN VII palsy
 Protein
 Cells
Season, recreational
activities, geography
Tick bite
MS-like lesions, CN and
nerve root enhancement
CMT
Hypertrophic
neuropathies
-
Established diagnostic
criteria
Gene testing +
nerve biopsy
Nerve root and plexus
thickening
MLD
Krabbe
-
-
arylsulfatase A
β-galacerebrosidase
-
CN + nerve root
enhancement early on
Neoplastic
Depends on
primary
 Protein
 Cells
Nodular enhancement
-
Image entire neural axis
Take-home messages
1. Pediatric nerve root enhancement =
nonspecific
2. Clinical presentation, imaging findings and
CSF testing will point towards the right
direction + guide further management
[email protected]
Suggested literature
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Zuccoli G et al. Redefining the Guillain-Barre spectrum in children: neuroimaging findings
of cranial nerve involvement. AJNR 2011;32(4):639-42.
Van Doorn PA et al. Clinical features, pathogenesis, and treatment of Guillain-Barre
syndrome. Lancet Neurol 2008;7:939-50.
Mulkey SB et al. Nerve root enhancement on spinal MRI in pediatric Guillain-Barre
syndrome. Pediatr Neurol 2010;43(4):263-9.
Vallat JM et al. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic
and therapeutic challenges for a treatable condition. Lancet Neurol 2010;9:402-12.
Sharma A et al. MR imaging of tubercular spinal arachnoiditis. AJR 1997;168(3):807-12.
Hildenbrand P et al. Lyme neuroborreliosis: manifestations of a rapidly emerging
zoonosis. AJNR 2009;30:1079-87.
Cellerini M et al. Imaging of the cauda equina in hereditary motor sensory neuropathies:
correlation with sural nerve biopsy. AJNR 2000;21:1793-8.
Morana G et al. Enhancing cranial nerves and cauda equina: an emerging magnetic
resonance pattern in metachromatic leukodystrophy and Krabbe disease.
Neuropediatrics 2009;40:291-4.
Zapadka M. Diffuse cauda equina nerve root enhancement. J Am Osteopath Coll Radiol
2012; Vol. 1, Issue 1.