spine problems that are actually nerve problems shawn jorgensen, md albany medical center aapm&r...

CANCEL SURGERY!

Spine problems that are actually

nerve problems

Shawn Jorgensen, MD

Albany Medical Center

AAPM&R Annual Assembly, October 2015

DISCLOSURES None

NON-SPINAL RADICULOPATHIES Radiculopathies are a frequent problem

200-350/100,000 (Shelerud 2002) The vast majority of radiculopathies are

spinal in origin

NON-SPINAL RADICULOPATHIES What about the patient without a clear

spinal cause on imaging and without an unusual risk factor for rarer cause?

What non-spinal causes are potentially hiding in the typical patient in your waiting room?

NON-SPINAL RADICULOPATHIES HIV

TBCryptococcusOther fungi

Syphilis Arachnoiditis Sarcoidosis GBS DM2 (Dumitru, 2002)

NON-SPINAL RADICULOPATHIES What about the patient without a clear

spinal cause on imaging and without an unusual risk factor for rarer cause? (1) Abnormal imaging, but mismatch (2) Essentially normal imaging

NON-SPINAL RADICULOPATHIES What if imaging isn’t normal, but there

is not a good correlation between imaging and clinical/EDX levels of pathology?Suspicious pathology but one level off

Right C4-5 HNP with compression of exiting rootShould be a right C5 radiculopathyClinically

Numbness right lateral shoulder/arm Weak deltoid, biceps

EDX NEE abnormalities in deltoid, biceps, rhomboid

major, paraspinals



Right C4-5 HNP with compression of exiting rootShould be a right C5 radiculopathyClinically

Numbness right thumb, index finger Weak wrist extension

EDX NEE abnormalities in pronator teres, ECRL,

paraspinals



Consider anomalous plexus anatomy Plexus can be:

Shifted (pre or postfixed)ExpandedContractedAltered in other ways

NON-SPINAL RADICULOPATHIES Plexus anomalies

Definitions Normal plexus anatomy

Large contribution from C5 and T1, occasional from C4 or T2

PrefixedLarge contribution from C4 with or without

small contribution from T1 Postfixed

Large contribution from T2 with or without small contribution from C5 (Pellerin 2010)

C4

C5

C6

C7

C8

C6

C7

C8

T1

T2

DorsalScapular(C6)

Suprascapular (C6-7)

Axillary (C6-7)

Ulnar (C8-T2)

DorsalScapular(C4)

Suprascapular (C4-5)

Axillary (C4-5)

Ulnar (C6-8)

Pre-fixedPost-fixed


Frequency Prefixed plexus

10-63%, average around 33%More common than postfixedMore common in women

Postfixed plexus0-72%, average around 15% (Pellerin 2010)


Variation in posterior (sensory) roots 40/40 had at least one, 33/40 at least two Most commonly between C6 and 7 Often process is thought to be one level higher

than it actually is (Perneczky 1980)

NON-SPINAL RADICULOPATHIES What if imaging is basically normal,

without any good anatomic (spinal) cause of the radiculopathy?

Consider non-spinal causes Infectious InflammatoryMalignantMotor neuron disease

NON-SPINAL RADICULOPATHIES Infections

Major consideration in immunocompromised In immunocompetent, otherwise healthy

patients they are not very commonPatients who have these infections rarely

present with isolated radicular symptomsRed flags:

Fever, chills, night sweats, unexplained weight loss, recent travel, history of infection (Shelerud 2002)


Lyme disease Multiorgan system disease caused by Borrelia

burgdorferi spirochete (bacteria) Carried by the vector Ixodes scapularis or Ixodes

pacifica Regionally specific: largely limited to northeastern

and nothern midwest USA Typical symptoms include fatigue, fever, rash

(erythema migrans) Neurological symptoms include peripheral (Bell’s

palsy, radiculopathy, mononeuropathy multiplex) and central (meningitis, encephalitis)

Non-neurological symptoms include AV block and arthritis


Lyme radiculopathy Will usually be in the setting of known disease

with other symptoms 6% of patients with Lyme disease (Brizzi 2014) When to suspect (Logigian 1997):

Exposure historyErythema migrans rash, other symptoms

consistent with Lyme diseaseNo history of diabetes, no rash of shingles, no

lab evidence of diabetes, VZV, EBV, CMV

NON-SPINAL RADICULOPATHIES Infectious

Lyme radiculopathy Often polyradiculopathy (Watson 2002)

Can be multifocal, involving an entire limb (Logigian 1997) or multiple limbs or regions (Logigian 1992)

Symptoms are often worse at night (Vallat 1987) Thoracic radiculopathy about 25%, often

involving multiple dermatomes (Pachner 1985)


Lyme radiculopathy Diagnosis

“Erythema migrans (aka. Erythema chronicum migrans or ECM) is the only manifestation of Lyme disease in the United States that is sufficiently distinctive to allow clinical diagnosis in the absence of laboratory confirmation.” (Wormser 2007 IDSA guidelines)

Erythemamigransrash

(bullseye)



Labs Early (Wormser 2007, IDSA guidelines)

“Serological testing is too insensitive in the acute phase, the first two weeks of infection, to be helpful diagnostically. Patients should be treated on the basis of clinical findings.”

If equivocal, both acute and convalescent (2 weeks after acute phase) serum samples should be tested



Labs Two-tiered approach (Wormser 2007, IDSA

guidelines)ELISA

If negative, no Lyme disease If positive or equivocal, same sample retested by

IgG and IgM Western Blot/Immunoblot Positive serology does not mean a given condition

is due to Lyme – reasonably high background seropositivity rate (4%)



CSF Often with pleocytosis Culture for B. burgdorferi PCR for amplification of B. burgdorferi gene

segments80% have combination of positive lyme

serology and western blot, lymphocytic pleocytosis in CSF, and CSF PCR or culture for B. burgdorferi (Logigian 1997)


Lyme radiculopathy Treatment (Wormser 2007, IDSA guidelines)

Early or late neurological disease LP? Ceftriaxone 2 grams daily for 14-28 days Chronic neurological symptoms

Response to treatment is slow and may be incomplete

Retreatment not recommended unless relapse is shown by reliable objective evidence

“There is no convincing biological evidence for the existence of symptomatic chronic B. burgdorferi infection among patients after receipt of recommended treatment regimens for Lyme disease” (Wormser 2007, IDSA guidelines)


Varicella-Zoster virus (VZV) Develop chickenpox as a child or are given

vaccineChickenpox <1 year of age increases risk of

shingles <60 years of age Virus then becomes latent and resides in dorsal

root ganglia or cranial nerve ganglia for life Reactivates with age (8-10x more common >60

years) or immunosupression Recurrence - <5% in immunocompetent (Gilden

2000)


Varicella-Zoster virus (VZV) Most common in the cranial nerves and thoracic

roots (Gilden 2000) Usually with a characteristic rash, but without –

Zoster sine herpete


Varicella-Zoster virus (VZV) Zoster sine herpete

Dermatomal distribution pain without rashTrue prevalence unknownDiagnosis

PCR of CSF or PMNs for amplification of VZV (and not HSV)

Peripheral antibodies are of no value (all positive), but antibodies in CSF are diagnostic (Gilden 2000)

Tends to recur (Gilden 1994)Treatment

IV acyclovir, PO famciclovir (Gilden 2000)


Varicella-Zoster virus (VZV) Usually only sensory complaints Occasionally weakness – Zoster paresis


Varicella-Zoster virus (VZV) Zoster paresis (Thomas 1972)

Not clear if this is spread to anterior root or anterior horn

Anterior horn cells have no natural immunityUsually middle aged and elderlyTiming

Always follows rash, from 1-5 weeks, usually within 2 weeks

All segments start simultaneously Once paresis begins, culminates in hours-days


Varicella-Zoster virus (VZV) Zoster paresis (Thomas 1972)

Distribution Right twice as common as left Most often cervical and lumbosacral Does not always coincide with sensory

distribution – can be widely separated One or two segments most common, but can be

regional, involving entire limbOutcome

>50% full recovery, 25% significant recovery

NON-SPINAL RADICULOPATHIES Inflammatory

Sarcoidosis Systemic autoimmune condition with predilection

of lymphatic, pulmonary, ocular systems Characterized by lymphadenopathy, anergy,

hypercalcemia, uveitis, skin lesions, pulmonary involvement

Neurosarcoidosis - ~5% of patients with sarcoid (Delaney 1977)1% of thoracic radiculopathies in one series

(Koffman 1998)22/23 neurological involvement was the

presenting/only complaint, usually CNSPNS involvement usually chronic (Delaney 1977)

NON-SPINAL RADICULOPATHIES Inflammatory

Sarcoidosis Diagnosis

CSF Pleocytosis Elevated protein Low glucose Negative cytology and culture (Atkinson 1982)

Angiotensin converting enzyme (ACE) level

NON-SPINAL RADICULOPATHIES Malignant

Can be from direct extension of primary tumor, metastases, or paraneoplastic

Direct extension more common in plexopathies (Watson 2002)

Radiculopathy usually from spinal or leptomeningeal spread (Watson 2002)


Leptomeningeal metastases (Watson 2002) Usually polyradicular Usually not the sentinel sign of recurrence, but

diagnosed in the setting of known metastases


Leptomeningeal metastases (Watson 2002) Diagnosis

MRI May show nodular, patchy enhancement or may

be negative


Leptomeningeal metastases (Watson 2002) Diagnosis

MRI May show nodular, patchy enhancement or may

be negativeLP

May require 3 separate, high volume taps to show cytology


Leptomeningeal metastases (Watson 2002) Prognosis

Poor Treatment

Palliative chemotherapy and intrathecal methotrexate may prolong survival by months

NON-SPINAL RADICULOPATHIES Motor neuron disease

Pure motor with no sensory involvementOften present as a radiculopathy

Anterior horn cell and pure motor root process – indistinguishable

ALS, PMA and Hirayama disease are most likely to present as a typical radiculopathy


Hirayama disease (aka Juvenile segmental SMA, Benign focal amyotrophy) (Amato 2008) Epidemiology

Usually between 15-25 years old Male>female Usually Asian descent

Clinical Progressive atrophy and weakness of hand and

forearm muscles for 6 years or less, then plateaus No sensory involvement No UMN signs 1/3 involve other limb clinically, more subclinically

with EDX abnormal “Cold paresis” – weakness is worse in the cold


ALS Primarily motor process – no sensory

involvement UMN and LMN signs in same patient in most

cases Presents focally, often subacutely

NON-SPINAL RADICULOPATHIES Polyradiculopathy (McGonagle 1990)

5% of all studies in EMG labEDX findings cannot generally distinguish

between different causesMost common cause is degenerative spine

processes, but several more ominous causes are in the differential

Subsequent studies separated them into Extradural Intradural / extraaxial Intraaxial

NON-SPINAL RADICULOPATHIES Polyradiculopathy (McGonagle 1990)

Extradural Majority were degenerative spine conditions Significantly older More pain, less weakness Slower progression Less disability CSF - increased protein was the only abnormality

Intradural/extraaxial Younger 1/3 with bowel or bladder issues Less pain Progressed faster CSF – usually established the diagnosis

NON-SPINAL RADICULOPATHIES Summary

When to suspect a non-spinal radiculopathy Imaging

Normal imagingAbnormalities on imaging don’t match

neurological level clinically or on EDX Neurological locations

PolyradiculopathyThoracic radiculopathy

Higher likelihood of systemic diseaseBackground systemic disease (cancer, infection,

autoimmune disease, immunosupressed)Systemic signs (fever, weight loss)

Pure motor symptoms


How to proceed when you encounter a likely non-spinal radiculopathy Rational workup

Imaging MRI – everyone without contraindication Contraindication – CT +/- myelogram


How to proceed when you encounter a likely non-spinal radiculopathy Rational workup

Signs of infection Lyme titers (Western blot if positive) PCR of PMNs for VZV LP

WBC, protein, glucoseCulture Lyme, viral culturesPCR for Lyme, VZV, HSVCytology (may need more than one)

ID consult Pure motor

More widespread EDX testing looking for signs of motor neuron disease

THANK YOU!!

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spine problems that are actually nerve problems shawn jorgensen, md albany medical center aapm&r...

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