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Spine problems that are actually nerve problems Shawn Jorgensen, MD Albany Medical Center AAPM&R Annual Assembly, October 2015 None Radiculopathies are a frequent problem 200-350/100,000 (Shelerud 2002) The vast majority of radiculopathies are spinal in origin 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? HIV TB Cryptococcus Other fungi Syphilis Arachnoiditis Sarcoidosis GBS DM2 (Dumitru, 2002) 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 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 root Should be a right C5 radiculopathy Clinically Numbness right lateral shoulder/arm Weak deltoid, biceps EDX NEE abnormalities in deltoid, biceps, rhomboid major, paraspinals 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 root Should be a right C5 radiculopathy Clinically Numbness right thumb, index finger Weak wrist extension EDX NEE abnormalities in pronator teres, ECRL, paraspinals 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 Consider anomalous plexus anatomy Plexus can be: Shifted (pre or postfixed) Expanded Contracted Altered in other ways Plexus anomalies Definitions Normal plexus anatomy Large contribution from C5 and T1, occasional from C4 or T2 Prefixed Large contribution from C4 with or without small contribution from T1 Postfixed Large contribution from T2 with or without small contribution from C5 (Pellerin 2010) Post-fixed Pre-fixed Dorsal Scapular (C6) (C4) C4 C6 Suprascapular (C6-7) (C4-5) C5 C7 C6 C8 C7 T1 C8 T2 Axillary (C6-7) (C4-5) Ulnar Ulnar (C6-8) (C8-T2) Plexus anomalies Frequency Prefixed plexus 10-63%, average around 33% More common than postfixed More common in women Postfixed plexus 0-72%, average around 15% (Pellerin 2010) Plexus anomalies 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) What if imaging is basically normal, without any good anatomic (spinal) cause of the radiculopathy? Consider non-spinal causes Infectious Inflammatory Malignant Motor neuron disease Infections Major consideration in immunocompromised In immunocompetent, otherwise healthy patients they are not very common Patients who have these infections rarely present with isolated radicular symptoms Red flags: Fever, chills, night sweats, unexplained weight loss, recent travel, history of infection (Shelerud 2002) Infections 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 Infections 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 history Erythema migrans rash, other symptoms consistent with Lyme disease No history of diabetes, no rash of shingles, no lab evidence of diabetes, VZV, EBV, CMV 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) Infectious 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) Erythema migrans rash (bullseye) Infectious Lyme radiculopathy Diagnosis 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 Infectious Lyme radiculopathy Diagnosis 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%) Infectious Lyme radiculopathy Diagnosis CSF Often with pleocytosis Culture for B. burgdorferi PCR for amplification of B. burgdorferi gene segments 80% have combination of positive lyme serology and western blot, lymphocytic pleocytosis in CSF, and CSF PCR or culture for B. burgdorferi (Logigian 1997) Infectious 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) Infectious Varicella-Zoster virus (VZV) Develop chickenpox as a child or are given vaccine Chickenpox <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) Infectious 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 Infectious Varicella-Zoster virus (VZV) Zoster sine herpete Dermatomal distribution pain without rash True prevalence unknown Diagnosis 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) Infectious Varicella-Zoster virus (VZV) Usually only sensory complaints Occasionally weakness – Zoster paresis Infectious 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 immunity Usually middle aged and elderly Timing Always follows rash, from 1-5 weeks, usually within 2 weeks All segments start simultaneously Once paresis begins, culminates in hours-days Infectious 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 limb Outcome >50% full recovery, 25% significant recovery 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 CNS PNS involvement usually chronic (Delaney 1977) Inflammatory Sarcoidosis Diagnosis CSF Pleocytosis Elevated protein Low glucose Negative cytology and culture (Atkinson 1982) Angiotensin converting enzyme (ACE) level 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) Malignant Leptomeningeal metastases (Watson 2002) Usually polyradicular Usually not the sentinel sign of recurrence, but diagnosed in the setting of known metastases Malignant Leptomeningeal metastases (Watson 2002) Diagnosis MRI May show nodular, patchy enhancement or may be negative Malignant Leptomeningeal metastases (Watson 2002) Diagnosis MRI May show nodular, patchy enhancement or may be negative LP May require 3 separate, high volume taps to show cytology Malignant Leptomeningeal metastases (Watson 2002) Prognosis Poor Treatment Palliative chemotherapy and intrathecal methotrexate may prolong survival by months Motor Pure motor with no sensory involvement Often present as a radiculopathy neuron disease Anterior horn cell and pure motor root process – indistinguishable ALS, PMA and Hirayama disease are most likely to present as a typical radiculopathy Motor neuron disease 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 Motor neuron disease ALS Primarily motor process – no sensory involvement UMN and LMN signs in same patient in most cases Presents focally, often subacutely Polyradiculopathy (McGonagle 1990) 5% of all studies in EMG lab EDX findings cannot generally distinguish between different causes Most common cause is degenerative spine processes, but several more ominous causes are in the differential Subsequent studies separated them into Extradural Intradural / extraaxial Intraaxial 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 Summary When to suspect a non-spinal radiculopathy Imaging Normal imaging Abnormalities on imaging don’t match neurological level clinically or on EDX Neurological locations Polyradiculopathy Thoracic radiculopathy Higher likelihood of systemic disease Background systemic disease (cancer, infection, autoimmune disease, immunosupressed) Systemic signs (fever, weight loss) Pure motor symptoms Summary How to proceed when you encounter a likely nonspinal radiculopathy Rational workup Imaging MRI – everyone without contraindication Contraindication – CT +/- myelogram Summary How to proceed when you encounter a likely nonspinal radiculopathy Rational workup Signs of infection Lyme titers (Western blot if positive) PCR of PMNs for VZV LP WBC, protein, glucose Culture Lyme, viral cultures PCR for Lyme, VZV, HSV Cytology (may need more than one) ID consult Pure motor More widespread EDX testing looking for signs of motor neuron disease Bibliography Atkinson BN, Ghelman B, Tsairis P, Warren RF, et al. 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