Amyotrophic lateral sclerosis differential diagnosis: Difference between revisions

Latest revision as of 17:50, 21 July 2021

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]Tarek Nafee, M.D. [3]

Overview

Amyotrophic lateral sclerosis is a neurodegenerative disease of upper and lower motor neurons. It is diagnosed clinically along with supportive electrophysiological testing. However, there is no specific biological marker that correlates with the disease pathology. The clinical diagnosis is straightforward in the majority of the cases, but false positive and false negative cases can arise early in the disease process. It usually occurs when only upper motor neurons or lower motor neurons signs and symptoms are present. It might be difficult to exclude ALS with only LMN signs with other spinal muscular atrophies, poliomyelitis, and Kennedy disease, etc.

^[1]

Differential Diagnosis

Conditions that are commonly mistaken for or difficult to differentiate from ALS are multifocal motor neuropathy with conduction block, cervical spondylotic myelopathy, Kennedy disease (KD), and Post-polio syndrome (PPS).^[2]^[3]^[4]^[3]^[4]

PPS presents with focal muscle weakness that very slowly progresses to other muscle groups over many years, and does not usually cause death. Patients who present with chronic respiratory muscle weakness should have a thorough evaluation to rule out ALS, as the onset of these symptoms are found in about 3% of ALS patients.^[5]^[4]

Diseases	History and Physical								Diagnostic tests		Other Findings
Diseases	Motor Deficit	Sensory deficit	Cranial nerve Involvement	Autonomic dysfunction	Proximal/Distal/Generalized	Ascending/Descending/Systemic	Unilateral (UL) or Bilateral (BL) or No Lateralization (NL)	Onset	Lab or Imaging Findings	Specific test	Other Findings
Adult Botulism	+	-	+	+	Generalized	Descending	BL	Sudden	Toxin test	Blood, Wound, or Stool culture	Diplopia, Hyporeflexia, Hypotonia, possible respiratory paralysis
Infant Botulism	+	-	+	+	Generalized	Descending	BL	Sudden	Toxin test	Blood, Wound, or Stool culture	Flaccid paralysis (Floppy baby syndrome), possible respiratory paralysis
Guillian-Barre syndrome^[6]	+	-	-	-	Generalized	Ascending	BL	Insidious	CSF: ↑Protein ↓Cells	Clinical & Lumbar Puncture	Progressive ascending paralysis following infection, possible respiratory paralysis
Eaton Lambert syndrome^[7]	+	-	+	+	Generalized	Systemic	BL	Intermittent	EMG, repetitive nerve stimulation test (RNS)	Voltage gated calcium channel (VGCC) antibody	Diplopia, ptosis, improves with movement (as the day progresses)
Myasthenia gravis^[8]	+	-	+	+	Generalized	Systemic	BL	Intermittent	EMG, Edrophonium test	Ach receptor antibody	Diplopia, ptosis, worsening with movement (as the day progresses)
Electrolyte disturbance^[9]	+	+	-	-	Generalized	Systemic	BL	Insidious	Electrolyte panel	↓Ca++, ↓Mg++, ↓K+	Possible arrhythmia
Organophosphate toxicity^[10]	+	+	-	+	Generalized	Ascending	BL	Sudden	Clinical diagnosis: physical exam & history	Clinical suspicion confirmed with RBC AchE activity	History of exposure to insecticide or living in farming environment. with : Diarrhea, Urination, Miosis, Bradycardia, Lacrimation, Emesis, Salivation, Sweating
Tick paralysis (Dermacentor tick)^[11]	+	-	-	-	Generalized	Ascending	BL	Insidious	Clinical diagnosis: physical exam & history	-	History of outdoor activity in Northeastern United States. The tick is often still latched to the patient at presentation (often in head and neck area)
Tetrodotoxin poisoning^[12]	+	-	+	+	Generalized	Systemic	BL	Sudden	Clinical diagnosis: physical exam & dietary history	-	History of consumption of puffer fish species.
Stroke^[13]	+/-	+/-	+/-	+/-	Generalized	Systemic	UL	Sudden	MRI +ve for ischemia or hemorrhage	MRI	Sudden unilateral motor and sensory deficit in a patient with a history of atherosclerotic risk factors (diabetes, hypertension, smoking) or atrial fibrillation.
Poliomyelitis^[14]	+	+	+	+/-	Proximal > Distal	Systemic	BL or UL	Sudden		PCR of CSF	Asymmetric paralysis following a flu-like syndrome.
Transverse myelitis^[15]	+	+	+	+	Proximal > Distal	Systemic	BL or UL	Sudden	MRI & Lumbar puncture	MRI	History of chronic viral or autoimmune disease (e.g. HIV)
Neurosyphilis^[16]^[17]	+	+	-	+/-	Generalized	Systemic	BL	Insidious	MRI & Lumbar puncture	CSF VDRL-specifc CSF FTA-Ab -sensitive^[18]	History of unprotected sex or multiple sexual partners. History of genital ulcer (chancre), diffuse maculopapular rash.
Muscular dystrophy^[19]	+	-	-	-	Proximal > Distal	Systemic	BL	Insidious	Genetic testing	Muscle biopsy	Progressive proximal lower limb weakness with calf pseudohypertrophy in early childhood. Gower sign positive.
Multiple sclerosis exacerbation^[20]	+	+	+	+	Generalized	Systemic	NL	Sudden	↑CSF IgG levels (monoclonal)	Clinical assessment and MRI ^[21]	Blurry vision, urinary incontinence, fatigue
Amyotrophic lateral sclerosis^[22]	+	-	-	-	Generalized	Systemic	BL	Insidious	Normal LP (to rule out DDx)	MRI & LP	Patient initially presents with upper motor neuron deficit (spasticity) followed by lower motor neuron deficit (flaccidity).
Inflammatory myopathy^[23]	+	-	-	-	Proximal > Distal	Systemic	UL or BL	Insidious	Elevated CK & Aldolase	Muscle biopsy	Progressive proximal muscle weakness in 3rd to 5th decade of life. With or without skin manifestations.

References

↑ Pradat PF, Bruneteau G (2006). "[Differential diagnosis and atypical subsets of amyotrophic lateral sclerosis]". Rev Neurol (Paris). 162 Spec No 2: 4S81–4S90. PMID 17128093.
↑ Pinto S, Swash M, de Carvalho M (2014). "Does surgery accelerate progression of amyotrophic lateral sclerosis?". J Neurol Neurosurg Psychiatry. 85 (6): 643–6. doi:10.1136/jnnp-2013-305770. PMID 23922387.
↑ ^3.0 ^3.1 Hardiman O, van den Berg LH, Kiernan MC (2011). "Clinical diagnosis and management of amyotrophic lateral sclerosis". Nat Rev Neurol. 7 (11): 639–49. doi:10.1038/nrneurol.2011.153. PMID 21989247.
↑ ^4.0 ^4.1 ^4.2
Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF; et al. (2015). "A comprehensive review of amyotrophic lateral sclerosis". Surg Neurol Int. 6: 171. doi:10.4103/2152-7806.169561. PMC 4653353. PMID 26629397.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
- Differentiating multifocal motor neuropathy from ALS is especially important, as patients with this neuropathy may benefit from intravenous immunoglobulin treatment, where ALS patients do not.<ref name="pmid20805527">Cats EA, van der Pol WL, Piepers S, Franssen H, Jacobs BC, van den Berg-Vos RM; et al. (2010). "Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy". Neurology. 75 (9): 818–25. doi:10.1212/WNL.0b013e3181f0738e. PMID 20805527.
↑ Pfeffer G, Povitz M, Gibson GJ, Chinnery PF (2015). "Diagnosis of muscle diseases presenting with early respiratory failure". J Neurol. 262 (5): 1101–14. doi:10.1007/s00415-014-7526-1. PMID 25377282.
↑ Talukder RK, Sutradhar SR, Rahman KM, Uddin MJ, Akhter H (2011). "Guillian-Barre syndrome". Mymensingh Med J. 20 (4): 748–56. PMID 22081202.
↑ Merino-Ramírez MÁ, Bolton CF (2016). "Review of the Diagnostic Challenges of Lambert-Eaton Syndrome Revealed Through Three Case Reports". Can J Neurol Sci. 43 (5): 635–47. doi:10.1017/cjn.2016.268. PMID 27412406.
↑ Gilhus NE (2016). "Myasthenia Gravis". N Engl J Med. 375 (26): 2570–2581. doi:10.1056/NEJMra1602678. PMID 28029925.
↑ Ozono K (2016). "[Diagnostic criteria for vitamin D-deficient rickets and hypocalcemia-]". Clin Calcium. 26 (2): 215–22. doi:CliCa1602215222 Check |doi= value (help). PMID 26813501.
↑ Kamanyire R, Karalliedde L (2004). "Organophosphate toxicity and occupational exposure". Occup Med (Lond). 54 (2): 69–75. PMID 15020723.
↑ Pecina CA (2012). "Tick paralysis". Semin Neurol. 32 (5): 531–2. doi:10.1055/s-0033-1334474. PMID 23677663.
↑ Bane V, Lehane M, Dikshit M, O'Riordan A, Furey A (2014). "Tetrodotoxin: chemistry, toxicity, source, distribution and detection". Toxins (Basel). 6 (2): 693–755. doi:10.3390/toxins6020693. PMC 3942760. PMID 24566728.
↑ Kuntzer T, Hirt L, Bogousslavsky J (1996). "[Neuromuscular involvement and cerebrovascular accidents]". Rev Med Suisse Romande. 116 (8): 605–9. PMID 8848683.
↑ Laffont I, Julia M, Tiffreau V, Yelnik A, Herisson C, Pelissier J (2010). "Aging and sequelae of poliomyelitis". Ann Phys Rehabil Med. 53 (1): 24–33. doi:10.1016/j.rehab.2009.10.002. PMID 19944665.
↑ West TW (2013). "Transverse myelitis--a review of the presentation, diagnosis, and initial management". Discov Med. 16 (88): 167–77. PMID 24099672.
↑ Liu LL, Zheng WH, Tong ML, Liu GL, Zhang HL, Fu ZG; et al. (2012). "Ischemic stroke as a primary symptom of neurosyphilis among HIV-negative emergency patients". J Neurol Sci. 317 (1–2): 35–9. doi:10.1016/j.jns.2012.03.003. PMID 22482824.
↑ Berger JR, Dean D (2014). "Neurosyphilis". Handb Clin Neurol. 121: 1461–72. doi:10.1016/B978-0-7020-4088-7.00098-5. PMID 24365430.
↑ Ho EL, Marra CM (2012). "Treponemal tests for neurosyphilis--less accurate than what we thought?". Sex Transm Dis. 39 (4): 298–9. doi:10.1097/OLQ.0b013e31824ee574. PMC 3746559. PMID 22421697.
↑ Falzarano MS, Scotton C, Passarelli C, Ferlini A (2015). "Duchenne Muscular Dystrophy: From Diagnosis to Therapy". Molecules. 20 (10): 18168–84. doi:10.3390/molecules201018168. PMID 26457695.
↑ Filippi M, Preziosa P, Rocca MA (2016). "Multiple sclerosis". Handb Clin Neurol. 135: 399–423. doi:10.1016/B978-0-444-53485-9.00020-9. PMID 27432676.
↑ Giang DW, Grow VM, Mooney C, Mushlin AI, Goodman AD, Mattson DH; et al. (1994). "Clinical diagnosis of multiple sclerosis. The impact of magnetic resonance imaging and ancillary testing. Rochester-Toronto Magnetic Resonance Study Group". Arch Neurol. 51 (1): 61–6. PMID 8274111.
↑ Riva N, Agosta F, Lunetta C, Filippi M, Quattrini A (2016). "Recent advances in amyotrophic lateral sclerosis". J Neurol. 263 (6): 1241–54. doi:10.1007/s00415-016-8091-6. PMC 4893385. PMID 27025851.
↑ Michelle EH, Mammen AL (2015). "Myositis Mimics". Curr Rheumatol Rep. 17 (10): 63. doi:10.1007/s11926-015-0541-0. PMID 26290112.

[pmid17128093-1] Pradat PF, Bruneteau G (2006). "[Differential diagnosis and atypical subsets of amyotrophic lateral sclerosis]". Rev Neurol (Paris). 162 Spec No 2: 4S81–4S90. PMID 17128093.

[pmid23922387-2] Pinto S, Swash M, de Carvalho M (2014). "Does surgery accelerate progression of amyotrophic lateral sclerosis?". J Neurol Neurosurg Psychiatry. 85 (6): 643–6. doi:10.1136/jnnp-2013-305770. PMID 23922387.

[pmid21989247-3] 3.0 ^3.1 Hardiman O, van den Berg LH, Kiernan MC (2011). "Clinical diagnosis and management of amyotrophic lateral sclerosis". Nat Rev Neurol. 7 (11): 639–49. doi:10.1038/nrneurol.2011.153. PMID 21989247.

[pmid26629397-4] 4.0 ^4.1 ^4.2 Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF; et al. (2015). "A comprehensive review of amyotrophic lateral sclerosis". Surg Neurol Int. 6: 171. doi:10.4103/2152-7806.169561. PMC 4653353. PMID 26629397.
Differentiating multifocal motor neuropathy from ALS is especially important, as patients with this neuropathy may benefit from intravenous immunoglobulin treatment, where ALS patients do not.<ref name="pmid20805527">Cats EA, van der Pol WL, Piepers S, Franssen H, Jacobs BC, van den Berg-Vos RM; et al. (2010). "Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy". Neurology. 75 (9): 818–25. doi:10.1212/WNL.0b013e3181f0738e. PMID 20805527.

[5] Differentiating multifocal motor neuropathy from ALS is especially important, as patients with this neuropathy may benefit from intravenous immunoglobulin treatment, where ALS patients do not.<ref name="pmid20805527">Cats EA, van der Pol WL, Piepers S, Franssen H, Jacobs BC, van den Berg-Vos RM; et al. (2010). "Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy". Neurology. 75 (9): 818–25. doi:10.1212/WNL.0b013e3181f0738e. PMID 20805527.

[pmid25377282-5] Pfeffer G, Povitz M, Gibson GJ, Chinnery PF (2015). "Diagnosis of muscle diseases presenting with early respiratory failure". J Neurol. 262 (5): 1101–14. doi:10.1007/s00415-014-7526-1. PMID 25377282.

[pmid22081202-6] Talukder RK, Sutradhar SR, Rahman KM, Uddin MJ, Akhter H (2011). "Guillian-Barre syndrome". Mymensingh Med J. 20 (4): 748–56. PMID 22081202.

[pmid27412406-7] Merino-Ramírez MÁ, Bolton CF (2016). "Review of the Diagnostic Challenges of Lambert-Eaton Syndrome Revealed Through Three Case Reports". Can J Neurol Sci. 43 (5): 635–47. doi:10.1017/cjn.2016.268. PMID 27412406.

[pmid28029925-8] Gilhus NE (2016). "Myasthenia Gravis". N Engl J Med. 375 (26): 2570–2581. doi:10.1056/NEJMra1602678. PMID 28029925.

[pmid26813501-9] Ozono K (2016). "[Diagnostic criteria for vitamin D-deficient rickets and hypocalcemia-]". Clin Calcium. 26 (2): 215–22. doi:CliCa1602215222 Check |doi= value (help). PMID 26813501.

[pmid15020723-10] Kamanyire R, Karalliedde L (2004). "Organophosphate toxicity and occupational exposure". Occup Med (Lond). 54 (2): 69–75. PMID 15020723.

[pmid23677663-11] Pecina CA (2012). "Tick paralysis". Semin Neurol. 32 (5): 531–2. doi:10.1055/s-0033-1334474. PMID 23677663.

[pmid24566728-12] Bane V, Lehane M, Dikshit M, O'Riordan A, Furey A (2014). "Tetrodotoxin: chemistry, toxicity, source, distribution and detection". Toxins (Basel). 6 (2): 693–755. doi:10.3390/toxins6020693. PMC 3942760. PMID 24566728.

[pmid8848683-13] Kuntzer T, Hirt L, Bogousslavsky J (1996). "[Neuromuscular involvement and cerebrovascular accidents]". Rev Med Suisse Romande. 116 (8): 605–9. PMID 8848683.

[pmid19944665-14] Laffont I, Julia M, Tiffreau V, Yelnik A, Herisson C, Pelissier J (2010). "Aging and sequelae of poliomyelitis". Ann Phys Rehabil Med. 53 (1): 24–33. doi:10.1016/j.rehab.2009.10.002. PMID 19944665.

[pmid24099672-15] West TW (2013). "Transverse myelitis--a review of the presentation, diagnosis, and initial management". Discov Med. 16 (88): 167–77. PMID 24099672.

[pmid22482824-16] Liu LL, Zheng WH, Tong ML, Liu GL, Zhang HL, Fu ZG; et al. (2012). "Ischemic stroke as a primary symptom of neurosyphilis among HIV-negative emergency patients". J Neurol Sci. 317 (1–2): 35–9. doi:10.1016/j.jns.2012.03.003. PMID 22482824.

[pmid24365430-17] Berger JR, Dean D (2014). "Neurosyphilis". Handb Clin Neurol. 121: 1461–72. doi:10.1016/B978-0-7020-4088-7.00098-5. PMID 24365430.

[pmid22421697-18] Ho EL, Marra CM (2012). "Treponemal tests for neurosyphilis--less accurate than what we thought?". Sex Transm Dis. 39 (4): 298–9. doi:10.1097/OLQ.0b013e31824ee574. PMC 3746559. PMID 22421697.

[pmid26457695-19] Falzarano MS, Scotton C, Passarelli C, Ferlini A (2015). "Duchenne Muscular Dystrophy: From Diagnosis to Therapy". Molecules. 20 (10): 18168–84. doi:10.3390/molecules201018168. PMID 26457695.

[pmid27432676-20] Filippi M, Preziosa P, Rocca MA (2016). "Multiple sclerosis". Handb Clin Neurol. 135: 399–423. doi:10.1016/B978-0-444-53485-9.00020-9. PMID 27432676.

[pmid8274111-21] Giang DW, Grow VM, Mooney C, Mushlin AI, Goodman AD, Mattson DH; et al. (1994). "Clinical diagnosis of multiple sclerosis. The impact of magnetic resonance imaging and ancillary testing. Rochester-Toronto Magnetic Resonance Study Group". Arch Neurol. 51 (1): 61–6. PMID 8274111.

[pmid27025851-22] Riva N, Agosta F, Lunetta C, Filippi M, Quattrini A (2016). "Recent advances in amyotrophic lateral sclerosis". J Neurol. 263 (6): 1241–54. doi:10.1007/s00415-016-8091-6. PMC 4893385. PMID 27025851.

[pmid26290112-23] Michelle EH, Mammen AL (2015). "Myositis Mimics". Curr Rheumatol Rep. 17 (10): 63. doi:10.1007/s11926-015-0541-0. PMID 26290112.

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@@ Line 1: / Line 1: @@
 __NOTOC__
-{{Amyotrophic lateral sclerosis}}
+[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Amyotrophic_lateral_sclerosis]]
-{{CMG}}; {{AE}}{{TarekNafee}}
+{{CMG}}; {{AE}}{{MMJ}}{{TarekNafee}}
 ==Overview==
-<nowiki>*</nowiki>Conditions that are commonly mistaken for or difficult to differentiate from ALS are multifocal motor neuropathy with conduction block, cervical spondylotic myelopathy, [[Kennedy disease]] (KD), and [[Post-polio syndrome|Post-polio syndrom]]<nowiki/>e (PPS). Differentiating multifocal motor neuropathy from ALS is especially important, as patients with this neuropathy may benefit from intravenous immunoglobulin treatment, where ALS patients do not. PPS presents with focal muscle weakness that very slowly progresses to other muscle groups over many years, and does not usually cause death. Patients who present with chronic respiratory muscle weakness should have a thorough evaluation to rule out ALS, as the onset of these symptoms are found in about 3% of ALS patients.
+Amyotrophic lateral sclerosis is a neurodegenerative disease of upper and lower motor neurons. It is diagnosed clinically along with supportive electrophysiological testing. However, there is no specific biological marker that correlates with the disease pathology. The clinical diagnosis is straightforward in the majority of the cases, but false positive and false negative cases can arise early in the disease process. It usually occurs when only [[upper motor neurons]] or [[lower motor neurons]] signs and symptoms are present. It might be difficult to exclude ALS with only LMN signs with other [[spinal muscular atrophies]], [[poliomyelitis]], and [[Kennedy disease]], etc.
+<ref name="pmid17128093">{{cite journal| author=Pradat PF, Bruneteau G| title=[Differential diagnosis and atypical subsets of amyotrophic lateral sclerosis]. | journal=Rev Neurol (Paris) | year= 2006 | volume= 162 Spec No 2 | issue=  | pages= 4S81-4S90 | pmid=17128093 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17128093  }} </ref>
 ==Differential Diagnosis==
-<nowiki>*</nowiki>Conditions that are commonly mistaken for or difficult to differentiate from ALS are multifocal motor neuropathy with conduction block, cervical spondylotic myelopathy, [[Kennedy disease]] (KD), and [[Post-polio syndrome|Post-polio syndrom]]<nowiki/>e (PPS).
-<nowiki>*</nowiki>Differentiating multifocal motor neuropathy from ALS is especially important, as patients with this neuropathy may benefit from intravenous immunoglobulin treatment, where ALS patients do not.
+*Conditions that are commonly mistaken for or difficult to differentiate from ALS are multifocal motor neuropathy with conduction block, cervical spondylotic myelopathy, [[Kennedy disease]] (KD), and [[Post-polio syndrome|Post-polio syndrom]]<nowiki/>e (PPS).<ref name="pmid23922387">{{cite journal| author=Pinto S, Swash M, de Carvalho M| title=Does surgery accelerate progression of amyotrophic lateral sclerosis? | journal=J Neurol Neurosurg Psychiatry | year= 2014 | volume= 85 | issue= 6 | pages= 643-6 | pmid=23922387 | doi=10.1136/jnnp-2013-305770 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23922387  }} </ref><ref name="pmid21989247">{{cite journal| author=Hardiman O, van den Berg LH, Kiernan MC| title=Clinical diagnosis and management of amyotrophic lateral sclerosis. | journal=Nat Rev Neurol | year= 2011 | volume= 7 | issue= 11 | pages= 639-49 | pmid=21989247 | doi=10.1038/nrneurol.2011.153 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21989247  }} </ref><ref name="pmid26629397">{{cite journal| author=Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF et al.| title=A comprehensive review of amyotrophic lateral sclerosis. | journal=Surg Neurol Int | year= 2015 | volume= 6 | issue=  | pages= 171 | pmid=26629397 | doi=10.4103/2152-7806.169561 | pmc=4653353 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26629397  }}
+*Differentiating multifocal motor neuropathy from ALS is especially important, as patients with this neuropathy may benefit from intravenous immunoglobulin treatment, where ALS patients do not.<nowiki><ref name="pmid20805527"></nowiki>{{cite journal| author=Cats EA, van der Pol WL, Piepers S, Franssen H, Jacobs BC, van den Berg-Vos RM et al.| title=Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy. | journal=Neurology | year= 2010 | volume= 75 | issue= 9 | pages= 818-25 | pmid=20805527 | doi=10.1212/WNL.0b013e3181f0738e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20805527  }}</ref><ref name="pmid21989247">{{cite journal| author=Hardiman O, van den Berg LH, Kiernan MC| title=Clinical diagnosis and management of amyotrophic lateral sclerosis. | journal=Nat Rev Neurol | year= 2011 | volume= 7 | issue= 11 | pages= 639-49 | pmid=21989247 | doi=10.1038/nrneurol.2011.153 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21989247  }} </ref><ref name="pmid26629397">{{cite journal| author=Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF et al.| title=A comprehensive review of amyotrophic lateral sclerosis. | journal=Surg Neurol Int | year= 2015 | volume= 6 | issue=  | pages= 171 | pmid=26629397 | doi=10.4103/2152-7806.169561 | pmc=4653353 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26629397  }} </ref>
+*PPS presents with focal muscle weakness that very slowly progresses to other muscle groups over many years, and does not usually cause death. Patients who present with chronic respiratory [[muscle]] weakness should have a thorough evaluation to rule out ALS, as the onset of these symptoms are found in about 3% of ALS patients.<ref name="pmid25377282">{{cite journal| author=Pfeffer G, Povitz M, Gibson GJ, Chinnery PF| title=Diagnosis of muscle diseases presenting with early respiratory failure. | journal=J Neurol | year= 2015 | volume= 262 | issue= 5 | pages= 1101-14 | pmid=25377282 | doi=10.1007/s00415-014-7526-1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25377282  }} </ref><ref name="pmid26629397">{{cite journal| author=Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF et al.| title=A comprehensive review of amyotrophic lateral sclerosis. | journal=Surg Neurol Int | year= 2015 | volume= 6 | issue=  | pages= 171 | pmid=26629397 | doi=10.4103/2152-7806.169561 | pmc=4653353 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26629397  }} </ref>
-<nowiki>*</nowiki>PPS presents with focal muscle weakness that very slowly progresses to other muscle groups over many years, and does not usually cause death. Patients who present with chronic respiratory [[muscle]] weakness should have a thorough evaluation to rule out ALS, as the onset of these symptoms are found in about 3% of ALS patients.
 {|
 |- style="background: #4479BA; color: #FFFFFF; text-align: center;"
@@ Line 32: / Line 36: @@
 No Lateralization (NL)</small>
 !<small>Onset</small>
 !<small>Lab or Imaging Findings</small>
 !<small>Specific  test</small>
 |-
-| style="background: #DCDCDC; padding: 5px; text-align: center;" | Adult Botulism
+| style="background: #DCDCDC; padding: 5px; text-align: center;" |Adult Botulism
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
 | style="background: #F5F5F5; padding: 5px; text-align:center" | -
@@ Line 46: / Line 50: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Sudden
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Toxin test
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Blood, Wound, or Stool culture
 | style="background: #F5F5F5; padding: 5px; text-align:center" |[[Diplopia]], [[Hyporeflexia|Hyporeflexia,]] [[Hypotonia]], possible respiratory paralysis
 |-
@@ Line 62: / Line 66: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |[[Flaccid paralysis]] ([[Floppy baby syndrome]]), possible respiratory paralysis
 |-
-| style="background: #DCDCDC; padding: 5px; text-align: center;" | [[Guillian-Barre syndrome]]<ref name="pmid22081202">{{cite journal| author=Talukder RK, Sutradhar SR, Rahman KM, Uddin MJ, Akhter H| title=Guillian-Barre syndrome. | journal=Mymensingh Med J | year= 2011 | volume= 20 | issue= 4 | pages= 748-56 | pmid=22081202 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22081202  }}</ref>
+| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Guillian-Barre syndrome]]<ref name="pmid22081202">{{cite journal| author=Talukder RK, Sutradhar SR, Rahman KM, Uddin MJ, Akhter H| title=Guillian-Barre syndrome. | journal=Mymensingh Med J | year= 2011 | volume= 20 | issue= 4 | pages= 748-56 | pmid=22081202 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22081202  }}</ref>
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
 | style="background: #F5F5F5; padding: 5px; text-align:center" | -
@@ Line 78: / Line 82: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Progressive [[ascending paralysis]] following infection, possible respiratory paralysis
 |-
-| style="background: #DCDCDC; padding: 5px; text-align: center;" | [[Eaton lambert syndrome|Eaton Lambert syndrome]]<ref name="pmid27412406">{{cite journal| author=Merino-Ramírez MÁ, Bolton CF| title=Review of the Diagnostic Challenges of Lambert-Eaton Syndrome Revealed Through Three Case Reports. | journal=Can J Neurol Sci | year= 2016 | volume= 43 | issue= 5 | pages= 635-47 | pmid=27412406 | doi=10.1017/cjn.2016.268 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27412406  }}</ref>
+| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Eaton lambert syndrome|Eaton Lambert syndrome]]<ref name="pmid27412406">{{cite journal| author=Merino-Ramírez MÁ, Bolton CF| title=Review of the Diagnostic Challenges of Lambert-Eaton Syndrome Revealed Through Three Case Reports. | journal=Can J Neurol Sci | year= 2016 | volume= 43 | issue= 5 | pages= 635-47 | pmid=27412406 | doi=10.1017/cjn.2016.268 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27412406  }}</ref>
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
 | style="background: #F5F5F5; padding: 5px; text-align:center" | -
@@ Line 87: / Line 91: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Intermittent
-| style="background: #F5F5F5; padding: 5px; text-align:center" | [[EMG]], repetitive nerve stimulation test (RNS)
+| style="background: #F5F5F5; padding: 5px; text-align:center" |[[EMG]], repetitive nerve stimulation test (RNS)
 | style="background: #F5F5F5; padding: 5px; text-align:center" |[[Voltage gated calcium channel|Voltage gated calcium channe]]<nowiki/>l<nowiki/> (VGCC) antibody
 | style="background: #F5F5F5; padding: 5px; text-align:center" |[[Diplopia]], [[ptosis]], improves with movement (as the day progresses)
@@ Line 100: / Line 104: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Intermittent
-| style="background: #F5F5F5; padding: 5px; text-align:center" | [[Electromyography|EMG]], [[Edrophonium|Edrophonium test]]
+| style="background: #F5F5F5; padding: 5px; text-align:center" |[[Electromyography|EMG]], [[Edrophonium|Edrophonium test]]
 | style="background: #F5F5F5; padding: 5px; text-align:center" |[[Acetylcholine receptor|Ach receptor]] antibody
 | style="background: #F5F5F5; padding: 5px; text-align:center" |[[Diplopia]], [[ptosis]], worsening with movement (as the day progresses)
@@ Line 113: / Line 117: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Insidious
-| style="background: #F5F5F5; padding: 5px; text-align:center" | Electrolyte panel
+| style="background: #F5F5F5; padding: 5px; text-align:center" |Electrolyte panel
 | style="background: #F5F5F5; padding: 5px; text-align:center" |↓Ca++, ↓Mg++, ↓K+
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Possible [[arrhythmia]]
@@ Line 126: / Line 130: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Sudden
-| style="background: #F5F5F5; padding: 5px; text-align:center" | Clinical diagnosis: physical exam & history
+| style="background: #F5F5F5; padding: 5px; text-align:center" |Clinical diagnosis: physical exam & history
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Clinical suspicion confirmed with RBC AchE activity
 | style="background: #F5F5F5; padding: 5px; text-align:center" |History of exposure to i[[Insecticide|nsecticide]] or living in farming environment. with : [[Diarrhea]], [[Urination]], [[Miosis]], [[Bradycardia]], [[Lacrimation]], [[Emesis]], [[Salivation]], [[Sweating]]
@@ Line 139: / Line 143: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Insidious
-| style="background: #F5F5F5; padding: 5px; text-align:center" | Clinical diagnosis: physical exam & history
+| style="background: #F5F5F5; padding: 5px; text-align:center" |Clinical diagnosis: physical exam & history
 | style="background: #F5F5F5; padding: 5px; text-align:center" | -
 | style="background: #F5F5F5; padding: 5px; text-align:center" |History of outdoor activity in Northeastern United States. The tick is often still latched to the patient at presentation (often in head and neck area)
@@ Line 152: / Line 156: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Sudden
-| style="background: #F5F5F5; padding: 5px; text-align:center" | Clinical diagnosis: physical exam & dietary history
+| style="background: #F5F5F5; padding: 5px; text-align:center" |Clinical diagnosis: physical exam & dietary history
 | style="background: #F5F5F5; padding: 5px; text-align:center" | -
-| style="background: #F5F5F5; padding: 5px; text-align:center" | History of consumption of puffer fish species.
+| style="background: #F5F5F5; padding: 5px; text-align:center" |History of consumption of puffer fish species.
 |-
 | style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Stroke]]<ref name="pmid8848683">{{cite journal| author=Kuntzer T, Hirt L, Bogousslavsky J| title=[Neuromuscular involvement and cerebrovascular accidents]. | journal=Rev Med Suisse Romande | year= 1996 | volume= 116 | issue= 8 | pages= 605-9 | pmid=8848683 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8848683  }}</ref>
@@ Line 165: / Line 169: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |UL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Sudden
-| style="background: #F5F5F5; padding: 5px; text-align:center" | MRI +ve for ischemia or hemorrhage
+| style="background: #F5F5F5; padding: 5px; text-align:center" |MRI +ve for ischemia or hemorrhage
 | style="background: #F5F5F5; padding: 5px; text-align:center" |MRI
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Sudden unilateral motor and sensory deficit in a patient with a history of [[Atherosclerosis|atherosclero]]<nowiki/>tic risk factors (diabetes, hypertension, smoking) or [[Atrial fibrillation|atrial fibrillation.]]
 |-
-| style="background: #DCDCDC; padding: 5px; text-align:center;" | [[Poliomyelitis]]<ref name="pmid19944665">{{cite journal| author=Laffont I, Julia M, Tiffreau V, Yelnik A, Herisson C, Pelissier J| title=Aging and sequelae of poliomyelitis. | journal=Ann Phys Rehabil Med | year= 2010 | volume= 53 | issue= 1 | pages= 24-33 | pmid=19944665 | doi=10.1016/j.rehab.2009.10.002 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19944665  }}</ref>
+| style="background: #DCDCDC; padding: 5px; text-align:center;" |[[Poliomyelitis]]<ref name="pmid19944665">{{cite journal| author=Laffont I, Julia M, Tiffreau V, Yelnik A, Herisson C, Pelissier J| title=Aging and sequelae of poliomyelitis. | journal=Ann Phys Rehabil Med | year= 2010 | volume= 53 | issue= 1 | pages= 24-33 | pmid=19944665 | doi=10.1016/j.rehab.2009.10.002 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19944665  }}</ref>
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
@@ Line 182: / Line 186: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Asymmetric paralysis following a flu-like syndrome.
 |-
-| style="background: #DCDCDC; padding: 5px; text-align:center;" | [[Transverse myelitis]]<ref name="pmid24099672">{{cite journal| author=West TW| title=Transverse myelitis--a review of the presentation, diagnosis, and initial management. | journal=Discov Med | year= 2013 | volume= 16 | issue= 88 | pages= 167-77 | pmid=24099672 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24099672  }}</ref>
+| style="background: #DCDCDC; padding: 5px; text-align:center;" |[[Transverse myelitis]]<ref name="pmid24099672">{{cite journal| author=West TW| title=Transverse myelitis--a review of the presentation, diagnosis, and initial management. | journal=Discov Med | year= 2013 | volume= 16 | issue= 88 | pages= 167-77 | pmid=24099672 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24099672  }}</ref>
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
@@ Line 220: / Line 224: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Insidious
-| style="background: #F5F5F5; padding: 5px; text-align:center" | Genetic testing
+| style="background: #F5F5F5; padding: 5px; text-align:center" |Genetic testing
 | style="background: #F5F5F5; padding: 5px; text-align:center" |[[Muscle biopsy]]
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Progressive proximal lower limb weakness with calf pseudohypertrophy in early childhood. [[Gowers' sign|Gower sign]] positive.
@@ Line 247: / Line 251: @@
 | style="background: #F5F5F5; padding: 5px; text-align:center" |BL
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Insidious
-| style="background: #F5F5F5; padding: 5px; text-align:center" | Normal [[Lumbar puncture|LP]] (to rule out DDx)
+| style="background: #F5F5F5; padding: 5px; text-align:center" |Normal [[Lumbar puncture|LP]] (to rule out DDx)
 | style="background: #F5F5F5; padding: 5px; text-align:center" |MRI & [[Lumbar puncture|LP]]
 | style="background: #F5F5F5; padding: 5px; text-align:center" |Patient initially presents with [[upper motor neuron]] deficit ([[spasticity]]) followed by [[lower motor neuron]] deficit ([[flaccidity]]).
 |-
-| style="background: #DCDCDC; padding: 5px; text-align:center;" | [[Myositis|Inflammatory myopathy]]<ref name="pmid26290112">{{cite journal| author=Michelle EH, Mammen AL| title=Myositis Mimics. | journal=Curr Rheumatol Rep | year= 2015 | volume= 17 | issue= 10 | pages= 63 | pmid=26290112 | doi=10.1007/s11926-015-0541-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26290112  }}</ref>
+| style="background: #DCDCDC; padding: 5px; text-align:center;" |[[Myositis|Inflammatory myopathy]]<ref name="pmid26290112">{{cite journal| author=Michelle EH, Mammen AL| title=Myositis Mimics. | journal=Curr Rheumatol Rep | year= 2015 | volume= 17 | issue= 10 | pages= 63 | pmid=26290112 | doi=10.1007/s11926-015-0541-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26290112  }}</ref>
 | style="background: #F5F5F5; padding: 5px; text-align:center" | +
 | style="background: #F5F5F5; padding: 5px; text-align:center" | -

Amyotrophic lateral sclerosis differential diagnosis: Difference between revisions

Latest revision as of 17:50, 21 July 2021

Overview

Differential Diagnosis

References

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