Pediatric spinal nerve root enhancement: Clinical and differential considerations Marinos Kontzialis...
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Transcript of Pediatric spinal nerve root enhancement: Clinical and differential considerations Marinos Kontzialis...
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
eEdE-200-6875
ASNR 53rd Annual Meeting, Chicago, April 25-30, [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 disruptionIs 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 1Transverse myelitis:
Quicker presentation compared to GBS (hours-few 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 demyelinationPrevalence: 1-7.7/100,000Clinically heterogeneous, grossly symmetric
sensory and/or motor neuropathyDevelops over > 8 weeks (GBS < 4 weeks)No preceding infectionCSF = increased protein, normal cell count
supportive of nerve root inflammation
Chronic inflammatory demyelinating polyneuropathy
CIDP may occur in the context of:Hepatitis CInflammatory bowel diseaseMonoclonal gammopathy of undetermined
significance (MGUS)HIV/AIDSOrgan transplantConnective 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 2-
week Hx of fever, lethargy, and emesis
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).
A B C
D
Case 3
CN VI, VII, VIII enhancement +
rim enhancing lesion in the right middle
cerebellar peduncle
Basilar meningitis CN V enhancement
A B C
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 VII + VIII enhancement
CN V enhancement
Bannwarth syndromeLyme meningo-radiculo-neuritis caused by spirochete
Borrelia burgdorferiMost common tick-borne disease in the USGeography, recreational habits, season (peak in the
summer) are clues!Confirmed by serum + CSF antibodiesCSF = lymphocytic meningitisErythema migrans in 89% of children = facilitates the
diagnosis
Bannwarth syndrome
MRI:MS-like periventricular white matter lesionsCranial nerve enhancementNerve 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 V thickening + enhancement
CN III thickening + enhancement
CN VII, VIII thickening + enhancement
Charcot-Marie-Tooth disease
Hereditary motor + sensory neuropathiesSymmetric + 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: MedulloblastomaGerminomaPineoblastomaPNET
EpendymomaAstrocytoma LymphomaLeukemia
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 tableDiagnosis 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 RashCN VII palsy
Protein Cells
Season, recreational activities, geography Tick bite MS-like lesions, CN and
nerve root enhancement
CMT Hypertrophic neuropathies - Established diagnostic
criteriaGene testing + nerve biopsy
Nerve root and plexus thickening
MLDKrabbe - - 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
Suggested literature1. Zuccoli G et al. Redefining the Guillain-Barre spectrum in children: neuroimaging findings
of cranial nerve involvement. AJNR 2011;32(4):639-42.2. Van Doorn PA et al. Clinical features, pathogenesis, and treatment of Guillain-Barre
syndrome. Lancet Neurol 2008;7:939-50.3. Mulkey SB et al. Nerve root enhancement on spinal MRI in pediatric Guillain-Barre
syndrome. Pediatr Neurol 2010;43(4):263-9.4. Vallat JM et al. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic
and therapeutic challenges for a treatable condition. Lancet Neurol 2010;9:402-12.5. Sharma A et al. MR imaging of tubercular spinal arachnoiditis. AJR 1997;168(3):807-12.6. Hildenbrand P et al. Lyme neuroborreliosis: manifestations of a rapidly emerging
zoonosis. AJNR 2009;30:1079-87.7. Cellerini M et al. Imaging of the cauda equina in hereditary motor sensory neuropathies:
correlation with sural nerve biopsy. AJNR 2000;21:1793-8.8. 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.
9. Zapadka M. Diffuse cauda equina nerve root enhancement. J Am Osteopath Coll Radiol 2012; Vol. 1, Issue 1.