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MR Imaging of Plexopathies Revisited
Poster No.:
C-0221
Congress:
ECR 2011
Type:
Educational Exhibit
Authors:
R. M. S. V. Vadapalli, M. AK; Hyderabad, AP/IN
Keywords:
Inflammation, Edema, Imaging sequences, Education, MR,
Neuroradiology peripheral nerve
DOI:
10.1594/ecr2011/C-0221
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Learning objectives
1.To discuss cardinal MR Imaging a Plexopathy
2.To illustrate vaious etiologies for brachial and lumbar plexopathies with clinical
examples.
3.Immune mediated plexopathies like CIDP,MMN,MADSAM which are plexo
neuropathies are hgihlighted with illustrative examples.
Background
The pathologic basis and histologic changes seen with brachial plexus lesions vary with
the underlying cause, which include compression, transection, ischemia, inflammation,
metabolic abnormalities, neoplasia, and radiation therapy.
Causes of Brachial Plexopathies
wide range of disease processes that may cause a brachial plexopathy,
radiation fibrosis, primary and metastatic lung cancer, and metastatic
breast cancer account for almost three-fourths of the causes. Radiation fibrosis, the
common cause, may occur several
months to years after the completion of therapy. Findings of radiation fibrosis include (a)
thickening and diffuse enhancement of the brachial
plexus without a focal mass and (b) soft-tissue changes with low signal intensity on both
T1- and T2-weighted images. Superior sullcus tumors at the lung apex may invade the
lower portion of the brachial plexus.
Many tumors may metastasize to the brachial plexus, causing a brachial plexopathy.
Breast cancer is the most likely to metastasize becausemajor lymphatic drainage routes
for the breast course through
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Radiation fibrosis
Breast cancer
Lung cancer
Benign tumors
Lymphangioma
Desmoid
Neurofibroma
Lipoma
Other malignant tumors
Neurofibrosarcoma
Ewing sarcoma
Eccrine sarcoma
Osteosarcoma
Auto immune/Immune mediated Plexopathies
CIDP(chronic inflammatory demyelinating polyneuropathy)
The symptoms and signs of CIDP may be asymmetrical
initially and have ascending involvements.
progresses slowly to symmetric weakness, loss of deep
tendon reflex, and impaired sensation in hands and
feet.
Antecedent infections can be identified far less
regularly in patients with CIDP than those with acute
inflammatory demyelinating polyneuropathy (AIDP).
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Elevated concentration of CSF protein and evidence of
demyelination on electrodiagnostic examination are
found in most patients with CIDP. The pathophysiology
of CIDP is still unclear, but an autoimmune mechanism
is proposed due to the likely responsiveness of
immune modulating treatments of CIDP.The
efficacy of corticosteroids plasma exchange and IVIg
MMN (multifocal motor neuropathy)
In contrast to CIDP, multifocal motor neuropathy
(MMN) shows an asymmetrical weakness and muscle
atrophy, typically in the distribution of individual peripheral
nerves without sensory involvement.
MMN
with a clinical picture of mononeuritis multiplex and
electrophysiologic evidence of persistent motor conduction
block (MCB) is considered an acquired
immune-mediated demyelinating motor polyneuropathy.
The diagnosis of MMN usually relies on the
presence of MCBs. High titers of anti-GM1 antibodies
are often detected in the serum of patients with
MMN.IVIg and cyclophosphamide are
effective treatment for the majority of patients with
MMN.
The major features that separate MADSAM neuropathy from
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typical CIDP are the electrophysiological findings and
favorable responses to plasma exchange, prednisone, or
IVIg. Similar to CIDP, CSF protein content is increased
in 60-80% of patients with MADSAM neuropathy.
On the other hand, polyclonal IgM antibodies against
GM1 may be detected in 40-80% of MMN patients, but
difficult to detect in CIDP and MADSAM neuropathy
MADSAM Neuropathy
Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is
characterized
by an asymmetric multifocal pattern of motor and sensory loss, and conduction block and
other features of
demyelination in nerve conduction studies. MADSAM neuropathy needs to be
differentiated from chronic
inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy
(MMN).
In classic CIDP, there are symmetric proximal and distal weakness, sensory deficit in both
upper and lower extremities and reduced deep tendon reflex(2). In MMN, limb weakness
without sensory loss is asymmetric in the distribution of individual peripheral nerves
and the weakness typically begins in the distal upper extremities. It is very important to
distinguish between CIDP, MADSAM neuropathy, and MMN by clinical, laboratory, and
histological features because of different effective therapeutic strategies.
EMNG findings of CIDP,MMN,MADSAM
In MADSAM neuropathy, MMN, and CIDP, the
nerve conduction studies show features of demyelination,
such as conduction block, temporal dispersion, prolonged
distal latencies, slow conduction velocities, and
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absent or prolonged F-wave latencies in one or more
motor nerves .MADSAM neuropathy is different from
MMN due to sensory nerves are involved and different
from CIDP due to conspicuous asymmetric multiple
nerves involvement.
Lumbar Plexopathies
Diabetic Plexopathy
Diabetic plexopathy typically affects the lumbosacral plexus more than a brachial.
It is distinguished from a peripheral polyneuropathy of long-standing diabetes by its
predominantly proximal symptoms. . Diagnosis is therefore based on presentation,
presence of diabetes, and the presence of acute electrodiagnostic findings to be
discussed later.
Traumatic Plexopathy
. Fractures or dislocations of the hip joint can produce traction injuries to the lumbosacral
plexus. Surgical intervention is imminent and requires initial periods of immobilization
post-operatively. The initial lesions can slowly recover during the post-operative
rehabilitation.
Hemorrhagic Plexopathy
Hemorrhagic plexopathy is usually caused in the retroperitoneal region, which can
compress on the plexus as it passes through either the iliac or psoas muscles. An
expanding hematoma within the more laterally located iliopsoas muscle can cause local
compression of the femoral nerve at the point along its course from its origin to the
inguinal ligament. A hemorrhagic plexopathy presents with severe groin pain radiating to
the anterior medial thigh and saphenous nerve territory. .
Neoplastic Plexopathy
Neoplastic plexopathy lesions originating in the pubic regions can invade the lumbosacral
plexus by direct expansion. The most common is a colorectal carcinoma., uterine,
prostatic, and ovarian tumors ,.Metastatic invasions of the retroperitoneum and the
lumbosacral plexus by breast, thyroid, testicular cancers, lymphomas, myelomas, and
melanomas are also well known. Patients with neurofibromatosis can develop grossly
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huge tumors involving any compartment of the plexus. Neoplastic plexopathies generally
present with unilateral pelvic pain and, when progressive, show lower motor neuron signs.
Radiation-Induced Lumbosacral Plexopathy
Radiation-induced lumbosacral plexopathy is similar to that affecting the brachial plexus.
Onset is difficult to determine, occurring from one to 31 years after radiation. Patients
receiving an external beam or internal cavity radiation are equally susceptible. Patients
generally present with slowly progressive, bilateral lower extremity weakness that tends
to affect the distal muscles more. Paresthesia and numbness are less frequently reported
in the initial symptoms.
Images for this section:
Fig. 1: MADSAM Neuropathy:EMNG In MADSAM neuropathy, MMN, and CIDP, the
nerve conduction studies show features of demyelination, such as conduction block,
temporal dispersion, prolonged distal latencies, slow conduction velocities, and absent
or prolonged F-wave latencies in one or more motor nerves .MADSAM neuropathy is
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different from MMN due to sensory nerves are involved and different from CIDP due to
conspicuous asymmetric multiple nerves involvement.
Fig. 2: MADSAM Neuropathy:EMNG
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Fig. 3: MADSAM Neuropathy:EMNG
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Fig. 4: MADSAM Neuropathy:EMNG
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Fig. 5: MADSAM Neuropathy:EMNG
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Fig. 6: CIDP Neuropathy:EMNG
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Fig. 7: CIDP Neuropathy:EMNG
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Fig. 8: CIDP Neuropathy:EMNG
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Fig. 9: CIDP Neuropathy:EMNG
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Fig. 10: In MADSAM neuropathy, MMN, and CIDP, the nerve conduction studies show
features of demyelination, such as conduction block, temporal dispersion, prolonged
distal latencies, slow conduction velocities, and absent or prolonged F-wave latencies in
one or more motor nerves .MADSAM neuropathy is different from MMN due to sensory
nerves are involved and different from CIDP due to conspicuous asymmetric multiple
nerves involvement.
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Fig. 11: MADSAM Neuropathy:EMNG
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Fig. 12: MADSAM Neuropathy:EMNG
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Fig. 13: MADSAM Neuropathy:EMNG
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Fig. 14: CIDP Neuropathy:EMNG
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Fig. 15: Nerve Biopsy :Inflammatory neuropathy:Sarcoid
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Fig. 16: Nerve Biopsy :Inflammatory neuropathy:Sarcoid
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Fig. 17: Nerve Biopsy :Inflammatory neuropathy:Sarcoid
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Fig. 18: Nerve Biopsy :Inflammatory neuropathy:Sarcoid
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Imaging findings OR Procedure details
Cardinal MR Imaging features of a Plexopathy
.Thickening of plexus
.pre ganglionic,ganglionic or post ganglionic segments of the nerve Root, trunks and
divisions is Present
. can be symmetrical(CIDP),Asymmetrical (MMN,MADSAM)
.Inflammation and fibrosis secondary to Radiation can occur
.Loss of Normal inter neural bundle fat planes
.Clumping and crowdin of roots and trunks
.T2 and STIR hyper intensity with nerve edema/Gliosis
.Presence of a Mass if the plexus is invaded ,with interruption,compression or splaying
.Patchy/heterogenous contrast enhancement
.
Findings of radiation fibrosis include (a) thickening and diffuse enhancement of the
brachial
plexus without a focal mass and (b) soft-tissue changes with low signal intensity on both
T1- and T2-weighted images. Superior sullcus tumors at the lung apex may invade the
lower portion of the brachial plexus.
In MADSAM neuropathy, MMN, and CIDP, the
nerve conduction studies show features of demyelination,
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such as conduction block, temporal dispersion, prolonged
distal latencies, slow conduction velocities, and
absent or prolonged F-wave latencies in one or more
motor nerves .MADSAM neuropathy is different from
MMN due to sensory nerves are involved and different
from CIDP due to conspicuous asymmetric multiple
nerves involvement.
Images for this section:
Fig. 1: Sarcoidosis Inflammatory brachial plexopathy Right
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Fig. 2: Sarcoidosis Inflammatory brachial plexopathy Right Normal Plexus on left
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Fig. 3: Sarcoidosis Inflammatory brachial plexopathy Right Normal Plexus on left
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Fig. 4: Lumbar Plexopathy with thickening of pre,post ganglionic segments of the roots
and lumbosacral trunks and sciatic nerves:Sarcoid
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Fig. 5: Lumbar Plexopathy with thickening of pre,post ganglionic segments of the roots
and lumbosacral trunks and sciatic nerves:Sarcoid
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Fig. 6: Brachial Plexopathy:Thoracic outlet syndrome
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Fig. 7: Brachial Plexopathy:Thoracic outlet syndrome
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Fig. 8: Brachial Plexopathy:Thoracic outlet syndrome
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Fig. 9: Brachial Plexopathy:Thoracic outlet syndrome
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Fig. 10: CIDP:Brachial Plexopathy symmetrical pattern
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Fig. 11: CIDP:Brachial Plexopathy symmetrical pattern
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Fig. 12: Brachial Plexopathy:Thoracic outlet syndrome
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Fig. 13: DTI with fiber tracking:Metastatic ca prostae with Lymphnodal masses
compressing the left lumbar Plexus
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Fig. 14: Contrast enhancement patterns of Plexopathy Lumbar (sarcoid)
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Fig. 15: Contrast enhancement patterns of Plexopathy Lumbar (sarcoid) Over view
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Fig. 16: Contrast enhancement patterns of Plexopathy Lumbar (sarcoid)
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Fig. 17: Contrast enhancement patterns of Plexo Neuro pathy Lumbar (sarcoid)
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Fig. 18: Lumbar Plexopathy with thickening of pre,post ganglionic segments of the roots
and lumbosacral trunks and sciatic nerves:Sarcoid Post contrast fat sat T1 weighted
images showing enhancement of roos and plexus
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Fig. 19: Lumbar Plexopathy with thickening of pre,post ganglionic segments of the roots
and lumbosacral trunks and sciatic nerves:Sarcoid Post contrast fat sat T1 weighted
images showing enhancement of roos and plexus
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Fig. 20: CIDP:Brachial Plexopathy symmetrical pattern 3D MIP
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Fig. 21: CIDP:Brachial Plexopathy symmetrical pattern 3D MIP
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Fig. 22: Aggressive Giant cell tumor Sacrum Infiltrating Sacral plexus with GS foraminal
extension on right:DTI with fiber tracking
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Fig. 23: Post Radiotherapy Ca Cervix with Radiation fibrosis,Lumboasacral
plexopathy,Pyriformis syndrome
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Fig. 24: Tracking exudate along scatic nerve and LS plexus Right from para spinal Koch's
Edema of pyriformis muscle.
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Fig. 25: Sacro iliac TB :Tracking exudate along scatic nerve and LS plexus Right
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Fig. 26: Sacro iliac TB inflammatory plexo neuropathy Right
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Fig. 27: CIDP:Lumbar Plexopathy symmetrical pattern 3D MIP
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Fig. 28: CIDP:Lumbar Plexopathy symmetrical pattern 3D MIP
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Fig. 29: CIDP:Lumbar Plexopathy symmetrical pattern 3D MIP
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Fig. 30: CIDP:Brachial Plexopathy symmetrical pattern 3D MIP
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Fig. 31: DTI with fiber tracking Aggressive Giant cell tumor Sacrum Infiltrating Sacral
plexus with GS foraminal extension on right:DTI with fiber tracking
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Fig. 32: POEMS SYNDROMEPolyneuropathy, organomegaly, endocrinopathy,
monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic
disease that occurs in the setting of a plasma cell dyscrasia.
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Fig. 33: POEM'S SYNDROME
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Fig. 34: POEMS SYNDROME Polyneuropathy, organomegaly, endocrinopathy,
monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic
disease that occurs in the setting of a plasma cell dyscrasia.
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Fig. 35: MADSAM :Multifocal acquired demyelinating sensory and motor (MADSAM)
neuropathy is characterized by an asymmetric multifocal pattern of motor and sensory
loss, and conduction block and other features of demyelination in nerve conduction
studies. MADSAM neuropathy needs to be differentiated from chronic inflammatory
demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN).
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Fig. 36: MADSAM :Multifocal acquired demyelinating sensory and motor (MADSAM)
neuropathy is characterized by an asymmetric multifocal pattern of motor and sensory
loss, and conduction block and other features of demyelination in nerve conduction
studies. MADSAM neuropathy needs to be differentiated from chronic inflammatory
demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN).
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Fig. 37: MADSAM :Multifocal acquired demyelinating sensory and motor (MADSAM)
neuropathy is characterized by an asymmetric multifocal pattern of motor and sensory
loss, and conduction block and other features of demyelination in nerve conduction
studies. MADSAM neuropathy needs to be differentiated from chronic inflammatory
demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN).
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Conclusion
MR Imaging features of Plexopathies and the spectrum of Imaging manifestations
in various etiologies,sub types involving brachial and lumbar plexuses or both are
highlighted in this educational exhibit
Personal Information
Rammohan Vadapalli
MD
Vijaya Diagnostics and research
Chippendale apartments,202
1-7-12
Musheerabad
Hyderabad
AP
India
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