Primary amyloidosis differential diagnosis: Difference between revisions

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==Differentiating Primary amyloidosis from Other Diseases==
==Differentiating Primary amyloidosis from Other Diseases==
{|
{|
! colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Organ System Involvement}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Causes}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF| Clinical Features}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Laboratory Findings}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Gold Standard Test}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Therapy}}
|-
| colspan="2" rowspan="6" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''P = Polyneuropathy'''''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''POEMS syndrome (Demyelinating)'''<ref name="pmid8608236">{{cite journal |vauthors=Gherardi RK, Bélec L, Soubrier M, Malapert D, Zuber M, Viard JP, Intrator L, Degos JD, Authier FJ |title=Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome |journal=Blood |volume=87 |issue=4 |pages=1458–65 |date=February 1996 |pmid=8608236 |doi= |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Monoclonal]] [[plasma cell]] proliferation
* Cytokine storm (IL-1, IL-6, IL-12, TNF alpha, VEGF)
| style="padding: 5px 5px; background: #F5F5F5;" |
* Symmetrical, ascending chronic progressive [[polyneuropathy]] with both [[Sensory system|sensory]] (pin-prick and vibration) and [[Motor skill|motor]] disability ([[Motor skill|motor]] > [[sensory]])
* Generalized/extermity [[pain]]
* [[Areflexia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*Increased number of [[Platelet|thrombocytes]]<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref><ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref><ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased number of [[Red blood cell|erythrocytes]]<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref><ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref><ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Elevated [[Cerebrospinal fluid|cerebrospinal fluid (CSF)]] [[protein]] content<ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref>
*Increased number of [[White blood cells|leukocytes]]<ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*High levels of [[Immunoglobulin G|IgG]] lambda or [[Immunoglobulin A|IgA]] lambda [[M protein|M-protein]] in the [[serum]]<ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased number of [[Plasma cell|plasma cells]] in the [[bone marrow]]<ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased serum [[Vascular endothelial growth factor|VEGF]] level<ref name="pmid19289745">{{cite journal |vauthors=Nobile-Orazio E, Terenghi F, Giannotta C, Gallia F, Nozza A |title=Serum VEGF levels in POEMS syndrome and in immune-mediated neuropathies |journal=Neurology |volume=72 |issue=11 |pages=1024–6 |date=March 2009 |pmid=19289745 |doi=10.1212/01.wnl.0000344569.13496.ff |url=}}</ref>
*Elevated levels of antitiroglobulin [[antibody]] and antithyroid peroxydase [[antibody]]<ref name="pmid25888197">{{cite journal |vauthors=Güneş HN, Bilecenoğlu NT, Şener U, Yoldaş TK |title=POEMS syndrome with peripheral and central nervous system demyelination: case report |journal=Neurologist |volume=19 |issue=4 |pages=101–3 |date=April 2015 |pmid=25888197 |doi=10.1097/NRL.0000000000000017 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[POEMS syndrome diagnostic criteria|International Myeloma Working Group (IMWG) clinical and laboratory diagnostic criteria]]<ref name="urlIMWG | International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma">{{cite web |url=http://imwg.myeloma.org/international-myeloma-working-group-imwg-criteria-for-the-diagnosis-of-multiple-myeloma/ |title=IMWG &#124; International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma |format= |work= |accessdate=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Metabolic syndrome|Metabolic Syndrome]] (Axonal pathology)'''<ref name="pmid25897354">{{cite journal |vauthors=Schreiber AK, Nones CF, Reis RC, Chichorro JG, Cunha JM |title=Diabetic neuropathic pain: Physiopathology and treatment |journal=World J Diabetes |volume=6 |issue=3 |pages=432–44 |date=April 2015 |pmid=25897354 |doi=10.4239/wjd.v6.i3.432 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Diabetes mellitus]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Symmetric sensorimotor distal polyneuropathy
* Asymmetric proximal neuropathy
* [[Oculomotor nerve palsy|3rd nerve palsy]]
* [[Carpal tunnel syndrome|Carpel tunnel syndrome]]
* [[Autonomic neuropathy]]
* "Glove and stocking" type pain
* [[Muscle wasting]]
* [[Hammer toe|Hammer toes]]
* [[Polyuria]]
* [[Polydipsia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Uncontrolled [[hyperglycemia]]
* Slowed [[Nerve conduction study|nerve conduction]]
* [[Small fiber peripheral neuropathy|Small fiber dysfunction]]
* [[Monofilament|Monofilament testing]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Fasting blood sugar|Fasting blood sugar level]] greater than equal to 126 mg/dl on 2 separate occasions<ref name="pmid20042775">{{cite journal |vauthors= |title=Diagnosis and classification of diabetes mellitus |journal=Diabetes Care |volume=33 Suppl 1 |issue= |pages=S62–9 |date=January 2010 |pmid=20042775 |doi=10.2337/dc10-S062 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Diabetes mellitus medical therapy|Anti-diabetic therapy]]
* [[Gabapentin]]
* [[Carbamazepine]]
* [[Foot care]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Vitamin deficiencies|Vitamin Deficiencies]] (Axonal Pathology)'''<ref name="pmid28129784">{{cite journal |vauthors=Ekabe CJ, Kehbila J, Abanda MH, Kadia BM, Sama CB, Monekosso GL |title=Vitamin B12 deficiency neuropathy; a rare diagnosis in young adults: a case report |journal=BMC Res Notes |volume=10 |issue=1 |pages=72 |date=January 2017 |pmid=28129784 |doi=10.1186/s13104-017-2393-3 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Vitamin B12 deficiency]] (Decreased [[S-Adenosyl methionine|S-adenosyl methionine]])<ref name="pmid2738712">{{cite journal |vauthors=Doi T, Kawata T, Tadano N, Iijima T, Maekawa A |title=Effect of vitamin B12 deficiency on S-adenosylmethionine metabolism in rats |journal=J. Nutr. Sci. Vitaminol. |volume=35 |issue=1 |pages=1–9 |date=February 1989 |pmid=2738712 |doi= |url=}}</ref>
* [[Thiamine deficiency|Vitamin B1 deficiency]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Primarily [[sensory]] deficits
* Vibration and [[proprioception]] affected
* [[Gait abnormality|Gait abnormalities]]
* [[Cognitive impairment]]
* [[Irritability]]
* [[Glossitis]]
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Anemia]] ([[Megaloblastic Anemias|megaloblastic]] in case of [[Vitamin B12 deficiency|B12 deficiency]])<ref name="pmid23262189">{{cite journal |vauthors=Berg RL, Shaw GR |title=Laboratory evaluation for vitamin B12 deficiency: the case for cascade testing |journal=Clin Med Res |volume=11 |issue=1 |pages=7–15 |date=February 2013 |pmid=23262189 |doi=10.3121/cmr.2012.1112 |url=}}</ref>
* Decreased [[serum]] [[Vitamin B12]] levels (< 200 pg/ml)
* [[Methylmalonic acidemia|Elevated methylmalonic acid]]
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Serum]] [[Vitamin B12]] levels<ref name="pmid2339684">{{cite journal |vauthors=Lindenbaum J, Savage DG, Stabler SP, Allen RH |title=Diagnosis of cobalamin deficiency: II. Relative sensitivities of serum cobalamin, methylmalonic acid, and total homocysteine concentrations |journal=Am. J. Hematol. |volume=34 |issue=2 |pages=99–107 |date=June 1990 |pmid=2339684 |doi= |url=}}</ref>
* [[Methylmalonic acid|Methylmalonic acid levels]]
* [[Intrinsic factor|Intrinsic factor antibodies]]<ref name="pmid232621892">{{cite journal |vauthors=Berg RL, Shaw GR |title=Laboratory evaluation for vitamin B12 deficiency: the case for cascade testing |journal=Clin Med Res |volume=11 |issue=1 |pages=7–15 |date=February 2013 |pmid=23262189 |pmc=3573090 |doi=10.3121/cmr.2012.1112 |url=}}</ref>
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Vitamin B12]] supplement ([[parenteral]])<ref name="pmid12086562">{{cite journal |vauthors=Lane LA, Rojas-Fernandez C |title=Treatment of vitamin b(12)-deficiency anemia: oral versus parenteral therapy |journal=Ann Pharmacother |volume=36 |issue=7-8 |pages=1268–72 |date=2002 |pmid=12086562 |doi=10.1345/aph.1A122 |url=}}</ref>
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Guillain-Barré syndrome|Guillain-Barre Syndrome]] (Demyelinating)'''<ref name="pmid117249122">{{cite journal |vauthors=Winer JB |title=Guillain Barré syndrome |journal=MP, Mol. Pathol. |volume=54 |issue=6 |pages=381–5 |date=December 2001 |pmid=11724912 |pmc=1187127 |doi= |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Anti-ganglioside antibodies|Anti-ganglioside]] and anti-[[myelin]] antibodies<ref name="pmid7486873">{{cite journal |vauthors=Rees JH, Gregson NA, Hughes RA |title=Anti-ganglioside GM1 antibodies in Guillain-Barré syndrome and their relationship to Campylobacter jejuni infection |journal=Ann. Neurol. |volume=38 |issue=5 |pages=809–16 |date=November 1995 |pmid=7486873 |doi=10.1002/ana.410380516 |url=}}</ref>
* [[Viral]] infections:<ref name="pmid3404161">{{cite journal |vauthors=Winer JB, Hughes RA, Anderson MJ, Jones DM, Kangro H, Watkins RP |title=A prospective study of acute idiopathic neuropathy. II. Antecedent events |journal=J. Neurol. Neurosurg. Psychiatry |volume=51 |issue=5 |pages=613–8 |date=May 1988 |pmid=3404161 |pmc=1033063 |doi= |url=}}</ref>
** [[Epstein Barr virus]]
** [[Human Immunodeficiency Virus (HIV)|HIV]]
** [[Cytomegalovirus]]
** [[Varicella Zoster Virus|Varicella Zoster virus]]
* [[Bacterial]] infections:<ref name="pmid16969154">{{cite journal |vauthors=Yuki N, Koga M |title=Bacterial infections in Guillain-Barré and Fisher syndromes |journal=Curr. Opin. Neurol. |volume=19 |issue=5 |pages=451–7 |date=October 2006 |pmid=16969154 |doi=10.1097/01.wco.0000245367.36576.e9 |url=}}</ref>
** [[Campylobacter]] infection
** [[Mycoplasma pneumoniae]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Rapid onset and quick progression
* Progression stops after 2-3 weeks
* Bilateral ascending [[Paraesthesia|paraesthesias]] and [[paralysis]] (generalized)
* [[Muscle weakness|Weakness]]
* [[Ataxia]]
* [[Areflexia]]
* No fever
* 4 sub-types:
**[[Acute inflammatory demyelinating polyneuropathy]]
**[[Acute motor axonal neuropathy]]
**Acute motor and sensory axonal neuropathy
**[[Miller Fisher syndrome]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Delayed F waves<ref name="pmid666277">{{cite journal |vauthors=Kimura J |title=Proximal versus distal slowing of motor nerve conduction velocity in the Guillain-Barré syndrome |journal=Ann. Neurol. |volume=3 |issue=4 |pages=344–50 |date=April 1978 |pmid=666277 |doi=10.1002/ana.410030412 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* Clinical diagnostic criteria (progressive weakness of more than two [[limbs]], [[areflexia]], and progression for no more than four weeks)<ref name="pmid11724912">{{cite journal |vauthors=Winer JB |title=Guillain Barré syndrome |journal=MP, Mol. Pathol. |volume=54 |issue=6 |pages=381–5 |date=December 2001 |pmid=11724912 |pmc=1187127 |doi= |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Intravenous]] [[immunoglobulins]]<ref name="pmid9014908">{{cite journal |vauthors= |title=Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group |journal=Lancet |volume=349 |issue=9047 |pages=225–30 |date=January 1997 |pmid=9014908 |doi= |url=}}</ref>
* [[Plasma]] exchange<ref name="pmid4022342">{{cite journal |vauthors= |title=Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group |journal=Neurology |volume=35 |issue=8 |pages=1096–104 |date=August 1985 |pmid=4022342 |doi= |url=}}</ref>
* [[Mechanical ventilation|Respiratory support]]
* [[DVT]]/[[PE]] [[prevention]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Chronic inflammatory demyelinating polyneuropathy|Chronic Inflammatory Demyelinating Polyneuropathy]] (CIDP) (Mixed axonal and demyelinatiing)'''<ref name="urlChronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network">{{cite web |url=https://jamanetwork.com/journals/jamaneurology/article-abstract/589258 |title=Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria &#124; JAMA Neurology &#124; JAMA Network |format= |work= |accessdate=}}</ref><ref name="urlonlinelibrary.wiley.com2">{{cite web |url=https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1468-1331.2009.02930.x |title=onlinelibrary.wiley.com |format= |work= |accessdate=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* Abnormal [[immune response]] (both [[Immunoglobulin G|IgG]] based [[Humoral immunity|humoral]] and [[Cell mediated immunity|T-Cell mediated]]) response to unknown [[antigen]] (possible culprits include [[myelin]] [[proteins]] P0, P2 and PMP22)<ref name="pmid2440998">{{cite journal |vauthors=Milner P, Lovelidge CA, Taylor WA, Hughes RA |title=P0 myelin protein produces experimental allergic neuritis in Lewis rats |journal=J. Neurol. Sci. |volume=79 |issue=3 |pages=275–85 |date=July 1987 |pmid=2440998 |doi= |url=}}</ref><ref name="pmid10713353">{{cite journal |vauthors=Gabriel CM, Gregson NA, Hughes RA |title=Anti-PMP22 antibodies in patients with inflammatory neuropathy |journal=J. Neuroimmunol. |volume=104 |issue=2 |pages=139–46 |date=May 2000 |pmid=10713353 |doi= |url=}}</ref><ref name="pmid10025777">{{cite journal |vauthors=Bouchard C, Lacroix C, Planté V, Adams D, Chedru F, Guglielmi JM, Said G |title=Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy |journal=Neurology |volume=52 |issue=3 |pages=498–503 |date=February 1999 |pmid=10025777 |doi= |url=}}</ref>
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* Slow onset and gradual progression
* [[Relapse|Relapsing]] and remitting course
* Symmetrical [[Proximal muscle weakness|proximal]] and [[Distal muscle weakness|distal motor]] and [[Sensory system|sensory weakness]] (legs>arms)
* [[Foot drop]]
* [[Numbness]], [[tingling]] and [[pain]]
* [[Areflexia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Elevated [[CSF]] [[protein]] ([[oligoclonal bands]] with normal [[White blood cells|WBCs]])<ref name="pmid11558784">{{cite journal |vauthors=Yan WX, Archelos JJ, Hartung HP, Pollard JD |title=P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy |journal=Ann. Neurol. |volume=50 |issue=3 |pages=286–92 |date=September 2001 |pmid=11558784 |doi= |url=}}</ref>
* Slowed [[Nerve conduction study|motor nerve conduction velocities]]<ref name="pmid2757528">{{cite journal |vauthors=Barohn RJ, Kissel JT, Warmolts JR, Mendell JR |title=Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria |journal=Arch. Neurol. |volume=46 |issue=8 |pages=878–84 |date=August 1989 |pmid=2757528 |doi= |url=}}</ref>
* Prolonged distal [[Motor skill|motor]] latencies (period between F wave and initial stimulation)
* Delayed F wave latencies (recorded from the [[feet]], hence called "F" waves)<ref name="pmid27575282">{{cite journal |vauthors=Barohn RJ, Kissel JT, Warmolts JR, Mendell JR |title=Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria |journal=Arch. Neurol. |volume=46 |issue=8 |pages=878–84 |date=August 1989 |pmid=2757528 |doi= |url=}}</ref>
* [[MRI]] contrast enhancement and enlargement of [[Vertebra|T2]] [[spinal segments]]<ref name="pmid23564314">{{cite journal |vauthors=Dimachkie MM, Barohn RJ |title=Chronic inflammatory demyelinating polyneuropathy |journal=Curr Treat Options Neurol |volume=15 |issue=3 |pages=350–66 |date=June 2013 |pmid=23564314 |pmc=3987657 |doi=10.1007/s11940-013-0229-6 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* EFNS/PNS criteria<ref name="urlonlinelibrary.wiley.com">{{cite web |url=https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1468-1331.2009.02930.x |title=onlinelibrary.wiley.com |format= |work= |accessdate=}}</ref>
* Koski criteria<ref name="pmid19091330">{{cite journal |vauthors=Koski CL, Baumgarten M, Magder LS, Barohn RJ, Goldstein J, Graves M, Gorson K, Hahn AF, Hughes RA, Katz J, Lewis RA, Parry GJ, van Doorn P, Cornblath DR |title=Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy |journal=J. Neurol. Sci. |volume=277 |issue=1-2 |pages=1–8 |date=February 2009 |pmid=19091330 |doi=10.1016/j.jns.2008.11.015 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Corticosteroids]]
* [[Intravenous immunoglobulin]] ([[IVIG]])
* [[Immunosupressive drug|Immunosupressants]] ([[Alemtuzumab|Alemtuzemab]] [[Azathioprine]] [[Cyclophosphamide]] [[Cyclosporine|Cyclosporin]] [[Etanercept]] [[Interferon-alpha]])
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Multifocal motor neuropathy|Multifocal Motor Neuropathy]]'''<ref name="RobbinsLawson2018">{{cite journal|last1=Robbins|first1=Nathaniel M|last2=Lawson|first2=Victoria|title=The Potential Misdiagnosis of Multifocal Motor Neuropathy as Amyotrophic Lateral Sclerosis—A Case Series|journal=US Neurology|volume=14|issue=2|year=2018|pages=102|issn=1758-4000|doi=10.17925/USN.2018.14.2.102}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Immune response|Abnormal immune response]] ([[Anti-ganglioside antibodies|Anti ganglioside]] [[Anti-ganglioside antibodies|GM-1]] [[IgM]] [[antibodies]])
| style="padding: 5px 5px; background: #F5F5F5;" |
* Progressive, asymmetric, distal and upper [[Limb (anatomy)|limb]] predominant weakness
* No significant [[sensory]] abnormalities
* [[Areflexia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Elevated [[CSF]] [[protein]]
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* Clinical criteria (EFNS/PNS):<ref name="pmid21199100">{{cite journal |vauthors= |title=European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision |journal=J. Peripher. Nerv. Syst. |volume=15 |issue=4 |pages=295–301 |date=December 2010 |pmid=21199100 |doi=10.1111/j.1529-8027.2010.00290.x |url=}}</ref>
** Slowly progressive or step-wise progressive, focal, asymmetric  [[limb]] weakness; i.e., [[Motor skill|motor]] involvement in the [[motor nerve]]  distribution of at least two nerves for  > 1 month.
** No objective [[Sensory system|sensory]]  abnormalities except  for minor vibration  sense abnormalities in  the [[lower limbs]]
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Intravenous immunoglobulin|Intravenous immunoglobulins]]
* [[Cyclophosphamide]]
* [[Rituximab]]
|-
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Organ System Involvement}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Organ System Involvement}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}

Revision as of 03:31, 30 October 2019

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Risk calculators and risk factors for Primary amyloidosis differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Differentiating Primary amyloidosis from Other Diseases

Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
P = Polyneuropathy POEMS syndrome (Demyelinating)[1]
Metabolic Syndrome (Axonal pathology)[8]
Vitamin Deficiencies (Axonal Pathology)[10]
Guillain-Barre Syndrome (Demyelinating)[16]
  • Clinical diagnostic criteria (progressive weakness of more than two limbs, areflexia, and progression for no more than four weeks)[21]
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)[24][25]
  • EFNS/PNS criteria[33]
  • Koski criteria[34]
Multifocal Motor Neuropathy[35]
  • Progressive, asymmetric, distal and upper limb predominant weakness
  • No significant sensory abnormalities
  • Areflexia
  • Clinical criteria (EFNS/PNS):[36]
    • Slowly progressive or step-wise progressive, focal, asymmetric limb weakness; i.e., motor involvement in the motor nerve distribution of at least two nerves for > 1 month.
    • No objective sensory abnormalities except for minor vibration sense abnormalities in the lower limbs
Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
Polyneuropathy POEMS syndrome (Demyelinating)
Metabolic Syndrome (Axonal pathology)
Vitamin Deficiencies (Axonal Pathology)
Guillain-Barre Syndrome (Demyelinating)
  • Delayed F waves
  • Clinical diagnostic criteria (progressive weakness of more than two limbs, areflexia, and progression for no more than four weeks)
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)
  • EFNS/PNS criteria
  • Koski criteria
Multifocal Motor Neuropathy
  • Progressive, asymmetric, distal and upper limb predominant weakness
  • No significant sensory abnormalities
  • Areflexia
  • Clinical criteria (EFNS/PNS):
    • Slowly progressive or step-wise progressive, focal, asymmetric limb weakness; i.e., motor involvement in the motor nerve distribution of at least two nerves for > 1 month.
    • No objective sensory abnormalities except for minor vibration sense abnormalities in the lower limbs
Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Organomegaly (Hepatosplenomegaly and Lymphadenopathy) Malaria
Kala-azar
Infective Hepatitis
Chronic Myelogenous Leukemia (CML)[37]
Lymphoma
Primary (AL) Amyloidosis
  • Typical green birefringence under polarized light after Congo red staining (appears in red under normal light)
  • Congo red staining
  • Melphalan-prednisone/dexamethasone
  • Dexamethasone plus Cyclophosphamide-thalidomide
  • Stem cell transplantation
Gaucher's Disease
Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Cardiac Failure
Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Plasma Cell Dyscrasias Multiple myeloma
  • Anemia
  • Thrombocytopenia
  • Leukopenia
  • Decreased albumin (reversed albumin:globulin ratio)
  • Increased serum creatinine, urea
  • Hypercalcemia
  • Elevated ESR
  • Normal-low alkaline phosphatase
  • RBC rouleaux formation
  • Bence-Jones proteins in urine
  • Clonal plasma cells on bone marrow exam greater than equal to 10%

AND

  • Any one of the following:
    • Evidence of end-organ damage
    • Hypercalcemia (>11 mg/dl)
    • Renal insufficiency
    • Anemia (Hb < 10 mg/dl)
    • Bone lesions
    • Greater than 1 lesions on MRI
Monoclonal gammopathy of undetermined significance (MGUS)
  • Serum M protein (IgG or IgA) <3g/dl

AND

  • Clonal bone marrow plasma cells < 10%

AND

  • No end-organ damage
  • Observation
Asymptomatic Plasma Cell Myeloma

(Smoldering and Indolent plasma cell myeloma)

  • Serum M protein (IgG or IgA greater than equal to 3 g/dl

OR

  • Urinary M protein greater than equal to 500 mg/24 h

AND/OR

  • Clonal bone marrow plasma cells 10-60%

AND

  • No end-organ damage
  • Observation
Plasmacytoma
  • On biopsy:
    • Solitary infiltrate of clonal plasma cells in bone (SBP) or soft tissue (EMP).
    • No evidence of infiltration by clonal plasma cells.
  • Negative skeletal survey plus MRI/CT spine and pelvis except for the solitary lesion.
  • Lack of hypercalcemia, renal insuffieciency, anemia, multiple bone lesions which would suggest MM
  • Diagnosis of exclusion
  • Radiotherapy
Skin Changes Scurvy
  1. ↑ Gherardi RK, Bélec L, Soubrier M, Malapert D, Zuber M, Viard JP, Intrator L, Degos JD, Authier FJ (February 1996). "Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome". Blood. 87 (4): 1458–65. PMID 8608236.
  2. ↑ 2.0 2.1 2.2 Nozza A (2017). "POEMS SYNDROME: an Update". Mediterr J Hematol Infect Dis. 9 (1): e2017051. doi:10.4084/MJHID.2017.051. PMC 5584767. PMID 28894560.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL (July 1980). "Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature". Medicine (Baltimore). 59 (4): 311–22. PMID 6248720.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Takatsuki K, Sanada I (September 1983). "Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases". Jpn. J. Clin. Oncol. 13 (3): 543–55. PMID 6315993.
  5. ↑ Nobile-Orazio E, Terenghi F, Giannotta C, Gallia F, Nozza A (March 2009). "Serum VEGF levels in POEMS syndrome and in immune-mediated neuropathies". Neurology. 72 (11): 1024–6. doi:10.1212/01.wnl.0000344569.13496.ff. PMID 19289745.
  6. ↑ Güneş HN, Bilecenoğlu NT, Şener U, Yoldaş TK (April 2015). "POEMS syndrome with peripheral and central nervous system demyelination: case report". Neurologist. 19 (4): 101–3. doi:10.1097/NRL.0000000000000017. PMID 25888197.
  7. ↑ "IMWG | International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma".
  8. ↑ Schreiber AK, Nones CF, Reis RC, Chichorro JG, Cunha JM (April 2015). "Diabetic neuropathic pain: Physiopathology and treatment". World J Diabetes. 6 (3): 432–44. doi:10.4239/wjd.v6.i3.432. PMID 25897354.
  9. ↑ "Diagnosis and classification of diabetes mellitus". Diabetes Care. 33 Suppl 1: S62–9. January 2010. doi:10.2337/dc10-S062. PMID 20042775.
  10. ↑ Ekabe CJ, Kehbila J, Abanda MH, Kadia BM, Sama CB, Monekosso GL (January 2017). "Vitamin B12 deficiency neuropathy; a rare diagnosis in young adults: a case report". BMC Res Notes. 10 (1): 72. doi:10.1186/s13104-017-2393-3. PMID 28129784.
  11. ↑ Doi T, Kawata T, Tadano N, Iijima T, Maekawa A (February 1989). "Effect of vitamin B12 deficiency on S-adenosylmethionine metabolism in rats". J. Nutr. Sci. Vitaminol. 35 (1): 1–9. PMID 2738712.
  12. ↑ Berg RL, Shaw GR (February 2013). "Laboratory evaluation for vitamin B12 deficiency: the case for cascade testing". Clin Med Res. 11 (1): 7–15. doi:10.3121/cmr.2012.1112. PMID 23262189.
  13. ↑ Lindenbaum J, Savage DG, Stabler SP, Allen RH (June 1990). "Diagnosis of cobalamin deficiency: II. Relative sensitivities of serum cobalamin, methylmalonic acid, and total homocysteine concentrations". Am. J. Hematol. 34 (2): 99–107. PMID 2339684.
  14. ↑ Berg RL, Shaw GR (February 2013). "Laboratory evaluation for vitamin B12 deficiency: the case for cascade testing". Clin Med Res. 11 (1): 7–15. doi:10.3121/cmr.2012.1112. PMC 3573090. PMID 23262189.
  15. ↑ Lane LA, Rojas-Fernandez C (2002). "Treatment of vitamin b(12)-deficiency anemia: oral versus parenteral therapy". Ann Pharmacother. 36 (7–8): 1268–72. doi:10.1345/aph.1A122. PMID 12086562.
  16. ↑ Winer JB (December 2001). "Guillain Barré syndrome". MP, Mol. Pathol. 54 (6): 381–5. PMC 1187127. PMID 11724912.
  17. ↑ Rees JH, Gregson NA, Hughes RA (November 1995). "Anti-ganglioside GM1 antibodies in Guillain-Barré syndrome and their relationship to Campylobacter jejuni infection". Ann. Neurol. 38 (5): 809–16. doi:10.1002/ana.410380516. PMID 7486873.
  18. ↑ Winer JB, Hughes RA, Anderson MJ, Jones DM, Kangro H, Watkins RP (May 1988). "A prospective study of acute idiopathic neuropathy. II. Antecedent events". J. Neurol. Neurosurg. Psychiatry. 51 (5): 613–8. PMC 1033063. PMID 3404161.
  19. ↑ Yuki N, Koga M (October 2006). "Bacterial infections in Guillain-Barré and Fisher syndromes". Curr. Opin. Neurol. 19 (5): 451–7. doi:10.1097/01.wco.0000245367.36576.e9. PMID 16969154.
  20. ↑ Kimura J (April 1978). "Proximal versus distal slowing of motor nerve conduction velocity in the Guillain-Barré syndrome". Ann. Neurol. 3 (4): 344–50. doi:10.1002/ana.410030412. PMID 666277.
  21. ↑ Winer JB (December 2001). "Guillain Barré syndrome". MP, Mol. Pathol. 54 (6): 381–5. PMC 1187127. PMID 11724912.
  22. ↑ "Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group". Lancet. 349 (9047): 225–30. January 1997. PMID 9014908.
  23. ↑ "Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group". Neurology. 35 (8): 1096–104. August 1985. PMID 4022342.
  24. ↑ "Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network".
  25. ↑ "onlinelibrary.wiley.com".
  26. ↑ Milner P, Lovelidge CA, Taylor WA, Hughes RA (July 1987). "P0 myelin protein produces experimental allergic neuritis in Lewis rats". J. Neurol. Sci. 79 (3): 275–85. PMID 2440998.
  27. ↑ Gabriel CM, Gregson NA, Hughes RA (May 2000). "Anti-PMP22 antibodies in patients with inflammatory neuropathy". J. Neuroimmunol. 104 (2): 139–46. PMID 10713353.
  28. ↑ Bouchard C, Lacroix C, Planté V, Adams D, Chedru F, Guglielmi JM, Said G (February 1999). "Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy". Neurology. 52 (3): 498–503. PMID 10025777.
  29. ↑ Yan WX, Archelos JJ, Hartung HP, Pollard JD (September 2001). "P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy". Ann. Neurol. 50 (3): 286–92. PMID 11558784.
  30. ↑ Barohn RJ, Kissel JT, Warmolts JR, Mendell JR (August 1989). "Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria". Arch. Neurol. 46 (8): 878–84. PMID 2757528.
  31. ↑ Barohn RJ, Kissel JT, Warmolts JR, Mendell JR (August 1989). "Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria". Arch. Neurol. 46 (8): 878–84. PMID 2757528.
  32. ↑ Dimachkie MM, Barohn RJ (June 2013). "Chronic inflammatory demyelinating polyneuropathy". Curr Treat Options Neurol. 15 (3): 350–66. doi:10.1007/s11940-013-0229-6. PMC 3987657. PMID 23564314.
  33. ↑ "onlinelibrary.wiley.com".
  34. ↑ Koski CL, Baumgarten M, Magder LS, Barohn RJ, Goldstein J, Graves M, Gorson K, Hahn AF, Hughes RA, Katz J, Lewis RA, Parry GJ, van Doorn P, Cornblath DR (February 2009). "Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy". J. Neurol. Sci. 277 (1–2): 1–8. doi:10.1016/j.jns.2008.11.015. PMID 19091330.
  35. ↑ Robbins, Nathaniel M; Lawson, Victoria (2018). "The Potential Misdiagnosis of Multifocal Motor Neuropathy as Amyotrophic Lateral Sclerosis—A Case Series". US Neurology. 14 (2): 102. doi:10.17925/USN.2018.14.2.102. ISSN 1758-4000.
  36. ↑ "European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision". J. Peripher. Nerv. Syst. 15 (4): 295–301. December 2010. doi:10.1111/j.1529-8027.2010.00290.x. PMID 21199100.
  37. ↑ Thompson PA, Kantarjian HM, Cortes JE (October 2015). "Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015". Mayo Clin. Proc. 90 (10): 1440–54. doi:10.1016/j.mayocp.2015.08.010. PMC 5656269. PMID 26434969.
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