COVID-19-associated polyneuritis cranialis

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For COVID-19 frequently asked outpatient questions, click here
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Javaria Anwer M.D.[2]

Synonyms and Keywords: covid19 associated polyneuritis cranialis, SARS Cov2 associated polyneuritis cranialis, nCOV associated polyneuritis cranialis, coronavirus linked polyneuritis cranialis, covid linked polyneuritis cranialis, polyneuritis cranialis and coronavirus, polyneuritis cranialis and covid19, COVID-19 linked PNC.

Overview

Polyneuritis cranialis (PNC) literally means inflammation of the cranial nerves. It is a rare neurological disorder characterized by multiple cranial nerve palsies sparing the spinal cord. The disease is a Guillain-Barré syndrome-Miller Fisher syndrome interface. SARS-CoV-2 virus which causes COVID-19 is emerging as a neurotropic virus. The pathogenesis of polyneuritis cranials is characterized by the demyelination of lower cranial nerves. COVID-19-associated PNC must be differentiated from other diseases that cause bulbar weakness, facial weakness, and ophthalmoparesis. The diagnosis of PNC is clinical and confirmed by Nerve Conduction Studies (NCS). There is only one case of COVID-19 associated PNC reported so far (first six months of the COVID-19 pandemic). Fixation nystagmus, bilateral abducens palsy, impaired visual acuity, gaze palsy and loss of deep tendon reflexes has been observed with no gait pathology. Treatment with acetaminophen caused complete recovery within 2 weeks. As the disease is believed to be an immune response to COVID-19 infection, the prevention of COVID-19 itself is the most promising primary prevention strategy.

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating COVID-19-associated polyneuritis cranialis 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

Incidence

  • Till date (July 25th, 2020) as the first six months of the COVID-19 pandemic pass, a single case report of COVID-19 associated PNC narrates the rarity of the disease.[17]

Age

  • The only case of COVID-19 associated PNC was reported in a 39-year-old patient.[5]
  • In general the age of patients reported of having PNC due to other reasons ranges from 10 - 40 years.[6][18][19]

Race

  • The only case of COVID-19 associated PNC was reported from Madrid, Spain. The race of the patient has not been mentioned clearly in the report.[5]
  • Having a single case reported to date makes it difficult to comment on the racial predilection.

Gender

  • The only case of COVID-19 associated PNC was reported in a male.[5]
  • Data regarding gender distribution for PNC, in general, is not available. Most of the cases of PNC that have been reported clearly show a predilection for male gender.[6][18][19]

Risk Factors

  • In general more severe patients are likely to have neurologic symptoms.[3]
  • There are no established risk factors for COVID-19-associated polyneuritis cranials (PNC).

Screening

  • Currently, there are no recommended guidelines for the routine screening for COVID-19-associated polyneuritis cranials or COVID-19. Some countries use temperature monitoring as a screening tool. Certain companies have launched the Screening Tool but there are no formal guidelines. Click here for more information on COVID-19 screening. [20]

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

  • The diagnosis of GBS and MFS is confirmed by Nerve conduction studies (NCS).
  • A decreased amplitude shows nerve conduction pathology.
  • Although other reports mention decreased nerve conduction in PNC patients, COVID-19 associated PNC report did not show NCS studies. The report considers not having done NCS due to the pandemic, a limitation.[5][1]

History and Symptoms

Common Symptoms

Less Common Symptoms

Physical Examination

Neuromuscular exam

Laboratory Findings

  • A positive qualitative real-time oropharyngeal swab RT PCR COVID-19 test confirmed COVID-19 infection in the COVID-19 associated PNC case.[5]
  • The cerebrospinal fluid (CSF) examination of COVID-19 associated PNC case revealed:[5][22]
    1. Opening pressure was normal (normal range 8-15 mm Hg).
    2. WBC count was reported normal with all monocytes (normal range 0 - 5 WBCs all monocytes).
    3. CSF protein was a little high i.e, 62 mg/dl (normal range 15 to 60 mg/dl). CSF protein can also be normal.[15][16] A high CSF protein and normal cell counts can be described as albumino-cytologic dissociation and is seen in 67% PNC cases.[6][1]
    4. CSF glucose was normal (normal range 50-80 mg/dl). CSF glucose is normal in PNC cases.
    5. CSF cytology was normal. Cytology is normal in PNC cases.
    6. CSF cultures and serology were sterile and negative respectively as occurs in other PNC cases.
    7. CSF RT PCR for COVID-19 was found negative in the patient.
  • Anti-ganglioside GM-1 IgM and IgG antibody levels ( antiganglioside GQ1b and GD1b) should be checked.[5] The COVID-19 associated PNC patient reported could not get the planned laboratory tests done due to hospital saturation.
  • CBC and differential, ESR, CRP, BMP, cardiac enzymes were all normal expect leukopenia was observed.[16]
  • Serum electrolytes: Retrospective studies from Italy and China demonstrated an assocition between hypokalemia, hypomagnesemia and COVID-19 due to degradation of ACE 2 loss, diuretic and corticosteroid use. Hypokalemia may lead to life-threatening arrhythmias. Close monitoring of serum electrolyte levels is essential specially in ICU patients.[23][24]

Electrocardiogram

X-ray

Echocardiography or Ultrasound

CT scan

Axial postcontrast T1 weighted MRI sequence, with yellow arrow showing contrast enhancement of CN VI - Case courtesy of Dr Bruno Di Muzio, Radiopaedia.org, rID: 37607
  • There are no CT scan findings associated with COVID-19-associated polyneuritis cranialis.[5]
  • Chest CT scan may be helpful in suggesting other organ involvement in the COVID-19 which is a multi-organ disease.
  • The CT scan findings in COVID-19 can be viewed by clicking here.

MRI

  • There are no MRI findings reported in COVID-19-associated polyneuritis cranialis but the writer consider it a limitation to the study.[6]
  • MRI in such neuropathies demonstrates nerve enhancement.[25][26] The MRI shown is not a case of COVID-19 related PNC but is to give an example of nerve enhancement.
  • MRI may be helpful in suggesting other organ involvement in the COVID-19 which is a multi-organ disease.
  • The MRI findings in COVID-19 can be viewed by clicking here.

Other Imaging Findings

There are no other imaging findings associated with COVID-19-associated polyneuritis cranialis.

Other Diagnostic Studies

There diagnostic studies associated with COVID-19-associated polyneuritis cranialis (PNC) that can help in the diagnosis include:

Treatment

Medical Therapy

Surgery

  • Surgical intervention is not recommended for the management of COVID-19-associated polyneuritis cranialis.

Primary Prevention

  • As the disease is believed to be an immune response to COVID-19 infection, the prevention of COVID-19 itself is the most promising primary prevention strategy.
  • There have been rigorous efforts in order to develop a vaccine for novel coronavirus and several vaccines are in the later phases of trials.[30]
  • The only prevention for COVID-19 associated PNC is the prevention and early diagnosis of COVID-19 infection itself. According to the CDC, the measures include:[31]
    • Frequent hand-washing with soap and water for at least 20 seconds or using a alcohol based hand sanitizer with at least 60% alcohol. Alcohol here refers to ethanol, not methanol/ wood alcohol, as FDA warns against the use of methanol containing hand-wash.[32]
    • Staying at least 6 feet (about 2 arms’ length) from other people who do not live with you.
    • Covering your mouth and nose with a cloth face cover when around others and covering sneezes and coughs.
    • Cleaning and disinfecting.

Secondary Prevention

  • Contact tracing helps reduce the spread of the disease.[33]

References

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