Introduction
There is growing interest on coronavirus disease 2019 (COVID-19)-associated neurological
manifestations and the interplay of COVID-19 in patients with existing neurological
illness.[1] We wish to share our rare case of COVID-19-associated neurological manifestations
in a patient with amyotrophic lateral sclerosis (ALS). ALS is a neurodegenerative
disease with very scarce information suggesting how COVID-19 affects patients with
ALS.[2]
[3] Case reports of COVID-19 in ALS are handful in literature and none of them highlights
the acute deterioration in respiratory status with acute COVID-19 and the subsequent
complications of long COVID-19. To the best of our knowledge, this is the first such
reported case.
Case Report
A 40-year-old male with recently diagnosed ALS presented to the emergency department
(ED) with a history of fever and worsening breathlessness for the last 4 days. The
patient was followed up by neurology department for ALS in the last 6 months. He had
baseline quadriparesis and was bedridden; however, he had no bulbar involvement and
respiratory compromise with Revised Amyotrophic Lateral Sclerosis Functional Rating
Scale (ALSFRS-R) score of 27. He was on tablet riluzole. In the ED, he was conscious
but tachypneic and his vitals were as follows: pulse rate of 120 /min, blood pressure,
130/86 mm Hg, respiratory rate of 32/min, and oxygen saturation of 86% on room air.
He was started on oxygen therapy and reverse-transcription polymerase chain reaction
(RT-PCR) test for severe acute respiratory syndrome coronavirus 2 was done. However,
his respiratory distress worsened rapidly within few hours and he was intubated and
ventilated. As his RT-PCR test came out to be positive, he was shifted to intensive
care unit (ICU) of COVID-19 center. He received steroids, anticoagulation, and supportive
treatment as per institutional protocol. The initial chest X-ray was not suggestive
of any COVID-19 features and was normal ([Fig. 1]). The computed tomographic scan could not be done; however, serial chest X-rays
were done daily that did not show any radiological changes suggestive of COVID-19.
Respiratory deterioration in this patient was due to respiratory muscle weakness rather
than parenchymal abnormalities. He remained on ventilator due to poor respiratory
efforts for the next 7 days. Tracheostomy was done at the end of first week in anticipation
of possibility of prolonged respiratory support. Nutritional and physiotherapy support
was provided for the holistic care and recovery.
Fig. 1 Normal chest X-ray on day 1 of admission.
In second week of illness, patient developed high-grade fever. The recorded axillary
temperature was 104.6 F. There were no features of acute infection/sepsis or organ
failure other than fever. All sepsis markers including procalcitonin were normal.
All the inflammatory markers suggestive of severe COVID-19 were within the normal
limits. Noninfective causes of fever in ICU like deep vein thrombosis, transfusion
reaction, and acute cholecystitis were ruled out. There were no findings on ultrasonography
chest and abdomen. All cultures sent came out to be sterile. The possibility of drug
fever was considered and riluzole was withheld and patient managed with symptomatic
care. The fever resolved after stopping the drug. In due hospital course, the patient
developed pneumothorax ([Fig. 2]) and was managed promptly with chest tube drainage and supportive care. The course
of illness was further complicated by difficult weaning in this patient. In the third
week of ICU stay, the patient developed ventilator-associated pneumonia secondary
to Acinetobacter
baumannii infection. He was treated as per culture sensitivity; however, unfortunately, the
patient succumbed to illness.
Fig. 2 Chest X-ray showing pneumothorax (blue arrow) in patient with severe coronavirus
disease 2019 and amyotrophic lateral sclerosis with intercostal drain in situ.
It is important to understand how COVID-19 can affect patients with ALS especially
because respiratory compromise is common in ALS patients and can be further complicated
by the clinical course of COVID-19 that can lead to respiratory failure and need for
intubation.1,2 The literature is scarce on COVID-19 infection as a trigger to accelerate respiratory
weakness in patients on ALS1 and the association between two remains speculative. Similar to the reported cases,1 our case also highlights a possibility of COVID-19-accelerated disease progression
in ALS. However, the respiratory decline in our case occurred during the acute COVID-19
phase instead of post-COVID-19 phase as reported earlier.
Second, another learning point was regarding the drug riluzole. It has been documented
to cause hypersensitivity reaction but drug fever has not been documented. However,
in our case, the possibility of drug fever could not be ruled out.[3]
[4] Moreover, no literature on drug interaction between remdesivir and riluzole exists
and the same needs exploration.
Underlying neuromuscular condition and prolonged hospital course in background of
COVID-19 pose a challenging scenario. We need to be aware that these patients may
require prolonged ICU stay due to difficult weaning that has its own set of complications
including secondary infections and barotrauma. The risk of pneumothorax in COVID-19
has been well documented.[5]
[6]
This case adds to existent sparse literature on prolonged course of COVID-19 and its
complications in a patient of ALS. Reporting of such cases and compilation of a COVID-ALS
registry[7] can throw more light about COVID-19 in ALS.
