COVID-19-associated Miller-Fischer syndrome: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{ | {{SI}} | ||
{{CMG}}; {{AE}} {{Arash.M}} | {{CMG}}; {{AE}} {{Arash.M}}, {{Fs}} | ||
{{SK}} MFS, fisher syndrome | {{SK}} MFS, fisher syndrome | ||
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==Historical Perspective== | ==Historical Perspective== | ||
The first reported case of MFS with history of | * The first reported case of MFS with a history of [[COVID-19]] was detected in January 2020 in Shanghai, who was a middle-aged woman diagnosed with MFS presented with [[areflexia]], acute [[weakness]] in both legs and severe [[fatigue]]. | ||
* Further reports were announced by medical groups in Spain and the USA which presented neuro-ophtalmological symptoms. <ref><nowiki>{{</nowiki>https://n.neurology.org/content/early/2020/04/17/WNL.0000000000009619<nowiki>}}</nowiki></ref> | |||
==Classification== | ==Classification== | ||
MFS is a rare variant of [[Guillain-Barre syndrome]], characterized by [[ophtalmoplegia]], [[areflexia]] and [[ataxia]]. | * MFS is a rare variant of [[Guillain-Barre syndrome]], characterized by [[ophtalmoplegia]], [[areflexia]] and [[ataxia]]. | ||
==Pathophysiology== | ==Pathophysiology== | ||
MFS is related to dysfunction of third, | * [[Miller Fisher Syndrome]] (MFS) is related to dysfunction of third, fourth, and sixth [[cranial nerves]]. | ||
* A typical [[Serology|serological]] finding in patients with MFS and prior history of [[covid-19]] is antibodies against GQ1b [[ganglioside]], though negative test for [[antibodies]] does not rule out the [[diagnosis]]. | |||
* The presence of [[ophthalmoparesis]] in MFS is related to a action of anti-GQ1b [[antibodies]] on the [[neuromuscular junction]] between the [[cranial nerves]] and [[Ocular muscles|ocular muscle]]. [[ELISA test]] is positive in 70% to 90% of patients.<ref><nowiki>{{</nowiki>https://pubmed.ncbi.nlm.nih.gov/10695710<nowiki>}}</nowiki></ref> | |||
==Causes== | ==Causes== | ||
Although MFS has been detected in some patients with | |||
*[[Influenza | * Although [[Miller Fisher Syndrome]] (MFS) has been detected in some patients with [[COVID-19]], other viral and [[bacterial]] infections can also cause MFS: | ||
*[[Cytomegalovirus]] | **[[Influenza Virus]] | ||
*[[Zika virus]] | **[[Cytomegalovirus]] | ||
*[[Mycoplasma]] | **[[Zika virus]] | ||
*[[Campylobacter]] | **[[Mycoplasma]] | ||
**[[Campylobacter]] | |||
==Differentiating COVID-19-associated Miller-Fischer syndrome from other Diseases== | ==Differentiating COVID-19-associated Miller-Fischer syndrome from other Diseases== | ||
*For further information about the differential diagnosis, [[COVID-19-associated Miller-Fischer syndrome differential diagnosis|click here]]. | |||
* To view the differential diagnosis of COVID-19, [[COVID-19 differential diagnosis|click here]]. | |||
==Epidemiology and Demographics== | |||
* While the [[incidence]] of MFS is one or two-person per million each year, the [[prevalence]] of MFS associated with [[COVID-19]] is still unknown. | |||
==Risk Factors== | |||
* There are no established [[Risk factor|risk factors]] for MFS associated with [[COVID-19]]. | |||
==Screening== | |||
* There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for patients with MFS caused by [[COVID-19]]. | |||
== | ==Natural History, Complications, and Prognosis== | ||
* There is an increased risk of death in patients over the age of 60-year-old. Hence, the [[mortality rate]] is estimated to be 3.6%. | |||
There is an increased risk of death in patients over the age of 60 year-old. Hence, the mortality rate is estimated to be 3.6%. | * Risk factors for severe illness and poor [[prognosis]] include: | ||
**[[Old age]] | |||
* Old age | ** Male gender | ||
* Male gender | ** Patients with | ||
* Patients with | ***[[Diabetes Mellitus]] | ||
** [[Diabetes Mellitus]] | ***[[Hypertension]] | ||
** [[Hypertension]] | ***[[COPD]] | ||
** [[COPD]] | ***[[CKD]] | ||
** [[CKD]] | |||
==Diagnosis== | ==Diagnosis== | ||
===Diagnostic Study of Choice=== | ===Diagnostic Study of Choice=== | ||
Although the diagnosis of [[ | |||
* Although the diagnosis of [[COVID-19]] is based on [[respiratory]] symptoms, it can be associated with [[neurological]] symptoms, which overlap the diagnosis of MFS. | |||
* Consequently, inpatient with prior history of [[COVID-19]], other [[Neurological|neurologic]] diseases should be ruled out and anti-GQ1b [[antibody]] test should be considered. | |||
* Also, in new patients with suspicious symptoms for [[COVID-19]] and neurological symptoms, a nasal swab test and neurological examination should be considered. | |||
* [[Magnetic resonance imaging|MRI]] may be performed as a part of the diagnostic workup. Although in majority of cases no abnormality is detected, enlargement and prominent enhancement in orbits and retro-orbital region has been reported in some cases.<ref><nowiki>{{</nowiki>http://www.ajnr.org/content/early/2020/05/28/ajnr.A6609<nowiki>}}</nowiki></ref>. <ref><nowiki>{{</nowiki>https://rarediseases.org/rare-diseases/miller-fisher-syndrome/<nowiki>}}</nowiki></ref> | |||
===History and Symptoms=== | ===History and Symptoms=== | ||
<ref><nowiki>{{</nowiki>http://www.ajnr.org/content/early/2020/05/28/ajnr.A6609<nowiki>}}</nowiki></ref> | ==== Common symptoms ==== | ||
* Common [[symptoms]] of covid-19 associated with [[MFS]] include: | |||
** [[Shortness of breath]] | |||
**[[Cough]] | |||
**[[Fever]] | |||
**[[Ophthalmoplegia]] | |||
**[[Areflexia]] | |||
**[[Ataxia]] | |||
==== Less common symptoms ==== | |||
* Less common symptoms of covid-19 associated with [[MFS]] include: | |||
** [[Headache]]<ref><nowiki>{{</nowiki>http://www.ajnr.org/content/early/2020/05/28/ajnr.A6609<nowiki>}}</nowiki></ref> | |||
**[[Diplopia]] | |||
**[[Blurred vision]] | |||
**[[Ptosis]] | |||
**[[Facial droop]] | |||
===Physical Examination=== | ===Physical Examination=== | ||
Patients with covid-19 associated with MFS present various signs and symptoms related to systematic and neurological presentation. Hence physical examination should be performed based on signs and symptoms include: | |||
* Patients with covid-19 associated with MFS present various signs and symptoms related to systematic and neurological presentation. Hence physical examination should be performed based on signs and symptoms include: | |||
====Vitals==== | ====Vitals==== | ||
Abnormal signs associated with covid-19: | Abnormal signs associated with covid-19: | ||
* [[Tachycardia]] | * [[Tachycardia]] | ||
* [[Tachypnea]] | * [[Tachypnea]] | ||
* Fever | *[[Fever]] | ||
==== | |||
====Neurological==== | |||
* [[Eye dropping]] | * [[Eye dropping]] | ||
* [[Blurry vision]] | * [[Blurry vision]] | ||
| Line 356: | Line 110: | ||
===Laboratory Findings=== | ===Laboratory Findings=== | ||
Laboratory findings consistent with the diagnosis of | |||
* Laboratory findings consistent with the diagnosis of COVID-19 include positive [[PCR]] nasal swab. | |||
* Ganglioside (GM1) Antibodies, IgG and IgM | * Laboratory tests for neurological signs are not diagnostic and should be used with other clinical parameters. They include: | ||
* GD1b Antibody, IgM | **[[Ganglioside]] (GM1) [[Antibodies]], [[IgG]] and [[IgM]] | ||
* GQ1b Antibody, IgG | ** GD1b [[Antibody]], [[IgM]] | ||
** GQ1b Antibody, [[IgG]] | |||
===Electrocardiogram=== | ===Electrocardiogram=== | ||
There are no ECG findings associated with | |||
* There are no [[The electrocardiogram|ECG]] findings associated with [[COVID-19]]. | |||
===X-ray=== | ===X-ray=== | ||
* [[Chest X-ray]] is less sensitive in detection of [[COVID-19]] in comparison with [[Computed tomography|CT]]. | |||
* However, in some cases [[lung consolidation]] and patchy peripheral opacities corresponding to [[ground glass opacities]] has been reported.<ref><nowiki>{{</nowiki>https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141645/<nowiki>}}</nowiki></ref> | |||
===Echocardiography or Ultrasound=== | ===Echocardiography or Ultrasound=== | ||
* Lung [[ultrasound]] may be helpful in the evaluation of patients with COVID-19. It indicates : | |||
* Multiple | |||
** Ranging from focal to diffuse with spared areas | * Multiple B-lines | ||
* Irregular and thickened pleural lines | ** Ranging from focal to diffuse with spared areas | ||
* Irregular and thickened [[pleural]] lines | |||
* Subpleural consolidations | * Subpleural consolidations | ||
* Alveolar consolidations | *[[Alveolar]] [[Consolidation (medicine)|consolidations]] | ||
* Bilateral [[A-lines]] | * Bilateral [[A-lines]] | ||
===CT scan=== | ===CT scan=== | ||
The preliminary findings of CT in COVID-19 associated with MFS include: | The preliminary findings of [[Computed tomography|CT]] in COVID-19 associated with MFS include: | ||
* Bilateral | * Bilateral ground glass opacities | ||
* Air space consolidation | * Air space [[Consolidation (medicine)|consolidation]] | ||
* Bronchovascular thickening | * Bronchovascular thickening | ||
* Traction bronchiectasis | * Traction [[bronchiectasis]] | ||
===MRI=== | ===MRI=== | ||
Brain MRI may be helpful in the diagnosis of MFS in patients with prior history of | |||
* Brain [[Magnetic resonance imaging|MRI]] may be helpful in the diagnosis of MFS in patients with prior history of COVID-19 and [[neurological]] manifestations. | |||
* Although there can be no abnormalities, multiple [[Cranial nerves|cranial nerve]] enhancement has been reported in some patients. | |||
===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
There are no other diagnostic studies associated with | |||
* There are no other diagnostic studies associated with COVID-19 with MFS manifestations. | |||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
No specific treatment and vaccine exists for covid-19 yet. However, patients with moderate to severe [[ARDS]] and respiratory manifestations can benefit from [[Mechanical ventilation]] and [[extracorporeal membrane oxygenation]] (ECMO). In some patients the combination of antiviral therapies like protease inhibitors, ritonavir, and lopinavir indicated partial success in treatment of | |||
* No specific treatment and vaccine exists for covid-19 yet. | |||
* However, patients with moderate to severe [[ARDS]] and respiratory manifestations can benefit from [[Mechanical ventilation]] and [[extracorporeal membrane oxygenation]] (ECMO). | |||
* In some patients the combination of antiviral therapies like [[protease inhibitors]], [[Ritonavir clinical pharmacology|ritonavir]], and [[lopinavir]] (100-400mg/day)<ref name="CaoWang2020">{{cite journal|last1=Cao|first1=Bin|last2=Wang|first2=Yeming|last3=Wen|first3=Danning|last4=Liu|first4=Wen|last5=Wang|first5=Jingli|last6=Fan|first6=Guohui|last7=Ruan|first7=Lianguo|last8=Song|first8=Bin|last9=Cai|first9=Yanping|last10=Wei|first10=Ming|last11=Li|first11=Xingwang|last12=Xia|first12=Jiaan|last13=Chen|first13=Nanshan|last14=Xiang|first14=Jie|last15=Yu|first15=Ting|last16=Bai|first16=Tao|last17=Xie|first17=Xuelei|last18=Zhang|first18=Li|last19=Li|first19=Caihong|last20=Yuan|first20=Ye|last21=Chen|first21=Hua|last22=Li|first22=Huadong|last23=Huang|first23=Hanping|last24=Tu|first24=Shengjing|last25=Gong|first25=Fengyun|last26=Liu|first26=Ying|last27=Wei|first27=Yuan|last28=Dong|first28=Chongya|last29=Zhou|first29=Fei|last30=Gu|first30=Xiaoying|last31=Xu|first31=Jiuyang|last32=Liu|first32=Zhibo|last33=Zhang|first33=Yi|last34=Li|first34=Hui|last35=Shang|first35=Lianhan|last36=Wang|first36=Ke|last37=Li|first37=Kunxia|last38=Zhou|first38=Xia|last39=Dong|first39=Xuan|last40=Qu|first40=Zhaohui|last41=Lu|first41=Sixia|last42=Hu|first42=Xujuan|last43=Ruan|first43=Shunan|last44=Luo|first44=Shanshan|last45=Wu|first45=Jing|last46=Peng|first46=Lu|last47=Cheng|first47=Fang|last48=Pan|first48=Lihong|last49=Zou|first49=Jun|last50=Jia|first50=Chunmin|last51=Wang|first51=Juan|last52=Liu|first52=Xia|last53=Wang|first53=Shuzhen|last54=Wu|first54=Xudong|last55=Ge|first55=Qin|last56=He|first56=Jing|last57=Zhan|first57=Haiyan|last58=Qiu|first58=Fang|last59=Guo|first59=Li|last60=Huang|first60=Chaolin|last61=Jaki|first61=Thomas|last62=Hayden|first62=Frederick G.|last63=Horby|first63=Peter W.|last64=Zhang|first64=Dingyu|last65=Wang|first65=Chen|title=A Trial of Lopinavir–Ritonavir in Adults Hospitalized with Severe Covid-19|journal=New England Journal of Medicine|volume=382|issue=19|year=2020|pages=1787–1799|issn=0028-4793|doi=10.1056/NEJMoa2001282}}</ref> indicated partial success in treatment of [[COVID-19]]. | |||
* [[Remdesivir]] (100-200mg/day)<ref name="urlCoronavirus disease 2019 (COVID-19) investigational drug information for remdesivir">{{cite web |url=https://reference.medscape.com/drug/remdesivir-4000090 |title=Coronavirus disease 2019 (COVID-19) investigational drug information for remdesivir |format= |work= |accessdate=}}</ref>, a drug originally developed to treat [[Ebola virus]], showed positive results against [[SARS-CoV-2]]. | |||
* [[Dexamethasone]] (6mg/day)<ref name="urlCorticosteroids | Coronavirus Disease COVID-19">{{cite web |url=https://www.covid19treatmentguidelines.nih.gov/immune-based-therapy/immunomodulators/corticosteroids/ |title=Corticosteroids | Coronavirus Disease COVID-19 |format= |work= |accessdate=}}</ref> has been announced as an effective treatment in patients with systematic manifestations. | |||
===Surgery=== | ===Surgery=== | ||
Surgical intervention is not recommended for the management of covid-19. | |||
* Surgical intervention is not recommended for the management of covid-19. | |||
===Primary Prevention=== | ===Primary Prevention=== | ||
Effective measures for the primary prevention of [[covid-19]] include | |||
* Effective measures for the [[primary prevention]] of [[covid-19]] include hand-washing, wearing of face masks, social distancing, avoidance of large gathering and self-isolation for patients who have mild symptoms. | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
[[Category:Up-To-Date]] | |||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
Latest revision as of 23:49, 12 December 2020
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Seyed Arash Javadmoosavi, MD[2], Fahimeh Shojaei, M.D.
Synonyms and keywords: MFS, fisher syndrome
Overview
Miller Fisher Syndrome (MFS) is an acute peripheral neuropathy that can develop after exposure to a viral or bacterial infection. It includes triad of ophthalmoplegia, areflexia and ataxia. In COVID-19 pandemic period, while COVID-19 typically presents with fever, shortness of breath (SOB) and respiratory symptoms, MFS with prior history of COVID-19 has been seen in several cases all around the world. One retrospective study in 214 patients has shown that 8.9 % of COVID-19 patients have reported peripheral neurological symptoms.
Historical Perspective
- The first reported case of MFS with a history of COVID-19 was detected in January 2020 in Shanghai, who was a middle-aged woman diagnosed with MFS presented with areflexia, acute weakness in both legs and severe fatigue.
- Further reports were announced by medical groups in Spain and the USA which presented neuro-ophtalmological symptoms. [1]
Classification
- MFS is a rare variant of Guillain-Barre syndrome, characterized by ophtalmoplegia, areflexia and ataxia.
Pathophysiology
- Miller Fisher Syndrome (MFS) is related to dysfunction of third, fourth, and sixth cranial nerves.
- A typical serological finding in patients with MFS and prior history of covid-19 is antibodies against GQ1b ganglioside, though negative test for antibodies does not rule out the diagnosis.
- The presence of ophthalmoparesis in MFS is related to a action of anti-GQ1b antibodies on the neuromuscular junction between the cranial nerves and ocular muscle. ELISA test is positive in 70% to 90% of patients.[2]
Causes
- Although Miller Fisher Syndrome (MFS) has been detected in some patients with COVID-19, other viral and bacterial infections can also cause MFS:
Differentiating COVID-19-associated Miller-Fischer syndrome from other Diseases
- For further information about the differential diagnosis, click here.
- To view the differential diagnosis of COVID-19, click here.
Epidemiology and Demographics
- While the incidence of MFS is one or two-person per million each year, the prevalence of MFS associated with COVID-19 is still unknown.
Risk Factors
- There are no established risk factors for MFS associated with COVID-19.
Screening
- There is insufficient evidence to recommend routine screening for patients with MFS caused by COVID-19.
Natural History, Complications, and Prognosis
- There is an increased risk of death in patients over the age of 60-year-old. Hence, the mortality rate is estimated to be 3.6%.
- Risk factors for severe illness and poor prognosis include:
- Old age
- Male gender
- Patients with
Diagnosis
Diagnostic Study of Choice
- Although the diagnosis of COVID-19 is based on respiratory symptoms, it can be associated with neurological symptoms, which overlap the diagnosis of MFS.
- Consequently, inpatient with prior history of COVID-19, other neurologic diseases should be ruled out and anti-GQ1b antibody test should be considered.
- Also, in new patients with suspicious symptoms for COVID-19 and neurological symptoms, a nasal swab test and neurological examination should be considered.
- MRI may be performed as a part of the diagnostic workup. Although in majority of cases no abnormality is detected, enlargement and prominent enhancement in orbits and retro-orbital region has been reported in some cases.[3]. [4]
History and Symptoms
Common symptoms
Less common symptoms
- Less common symptoms of covid-19 associated with MFS include:
Physical Examination
- Patients with covid-19 associated with MFS present various signs and symptoms related to systematic and neurological presentation. Hence physical examination should be performed based on signs and symptoms include:
Vitals
Abnormal signs associated with covid-19:
Neurological
- Eye dropping
- Blurry vision
- Paresthesia
- Decreased sensation
- Myalgia
- Weakness of breathing muscle
Laboratory Findings
- Laboratory findings consistent with the diagnosis of COVID-19 include positive PCR nasal swab.
- Laboratory tests for neurological signs are not diagnostic and should be used with other clinical parameters. They include:
- Ganglioside (GM1) Antibodies, IgG and IgM
- GD1b Antibody, IgM
- GQ1b Antibody, IgG
Electrocardiogram
X-ray
- Chest X-ray is less sensitive in detection of COVID-19 in comparison with CT.
- However, in some cases lung consolidation and patchy peripheral opacities corresponding to ground glass opacities has been reported.[6]
Echocardiography or Ultrasound
- Lung ultrasound may be helpful in the evaluation of patients with COVID-19. It indicates :
- Multiple B-lines
- Ranging from focal to diffuse with spared areas
- Irregular and thickened pleural lines
- Subpleural consolidations
- Alveolar consolidations
- Bilateral A-lines
CT scan
The preliminary findings of CT in COVID-19 associated with MFS include:
- Bilateral ground glass opacities
- Air space consolidation
- Bronchovascular thickening
- Traction bronchiectasis
MRI
- Brain MRI may be helpful in the diagnosis of MFS in patients with prior history of COVID-19 and neurological manifestations.
- Although there can be no abnormalities, multiple cranial nerve enhancement has been reported in some patients.
Other Diagnostic Studies
- There are no other diagnostic studies associated with COVID-19 with MFS manifestations.
Treatment
Medical Therapy
- No specific treatment and vaccine exists for covid-19 yet.
- However, patients with moderate to severe ARDS and respiratory manifestations can benefit from Mechanical ventilation and extracorporeal membrane oxygenation (ECMO).
- In some patients the combination of antiviral therapies like protease inhibitors, ritonavir, and lopinavir (100-400mg/day)[7] indicated partial success in treatment of COVID-19.
- Remdesivir (100-200mg/day)[8], a drug originally developed to treat Ebola virus, showed positive results against SARS-CoV-2.
- Dexamethasone (6mg/day)[9] has been announced as an effective treatment in patients with systematic manifestations.
Surgery
- Surgical intervention is not recommended for the management of covid-19.
Primary Prevention
- Effective measures for the primary prevention of covid-19 include hand-washing, wearing of face masks, social distancing, avoidance of large gathering and self-isolation for patients who have mild symptoms.
References
- ↑ {{https://n.neurology.org/content/early/2020/04/17/WNL.0000000000009619}}
- ↑ {{https://pubmed.ncbi.nlm.nih.gov/10695710}}
- ↑ {{http://www.ajnr.org/content/early/2020/05/28/ajnr.A6609}}
- ↑ {{https://rarediseases.org/rare-diseases/miller-fisher-syndrome/}}
- ↑ {{http://www.ajnr.org/content/early/2020/05/28/ajnr.A6609}}
- ↑ {{https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141645/}}
- ↑ Cao, Bin; Wang, Yeming; Wen, Danning; Liu, Wen; Wang, Jingli; Fan, Guohui; Ruan, Lianguo; Song, Bin; Cai, Yanping; Wei, Ming; Li, Xingwang; Xia, Jiaan; Chen, Nanshan; Xiang, Jie; Yu, Ting; Bai, Tao; Xie, Xuelei; Zhang, Li; Li, Caihong; Yuan, Ye; Chen, Hua; Li, Huadong; Huang, Hanping; Tu, Shengjing; Gong, Fengyun; Liu, Ying; Wei, Yuan; Dong, Chongya; Zhou, Fei; Gu, Xiaoying; Xu, Jiuyang; Liu, Zhibo; Zhang, Yi; Li, Hui; Shang, Lianhan; Wang, Ke; Li, Kunxia; Zhou, Xia; Dong, Xuan; Qu, Zhaohui; Lu, Sixia; Hu, Xujuan; Ruan, Shunan; Luo, Shanshan; Wu, Jing; Peng, Lu; Cheng, Fang; Pan, Lihong; Zou, Jun; Jia, Chunmin; Wang, Juan; Liu, Xia; Wang, Shuzhen; Wu, Xudong; Ge, Qin; He, Jing; Zhan, Haiyan; Qiu, Fang; Guo, Li; Huang, Chaolin; Jaki, Thomas; Hayden, Frederick G.; Horby, Peter W.; Zhang, Dingyu; Wang, Chen (2020). "A Trial of Lopinavir–Ritonavir in Adults Hospitalized with Severe Covid-19". New England Journal of Medicine. 382 (19): 1787–1799. doi:10.1056/NEJMoa2001282. ISSN 0028-4793.