Intensive reviews of his medical records from the local hospital and his medical history Figure 1 indicated that muscle weakness had started to spread from the lower to the upper limbs two days before admission to the local hospital, peaked on day 16 and continued to day A neurological examination revealed decreased positional sense in the lower limbs, depressed tendon reflexes, the presence of ciliary sign, and a bilateral abducens muscle disorder, suggesting palsy of the facial and bilateral abducens nerve.
Figure 1 Clinical course of Case 1. Thus, he was diagnosed with GBS accompanied by respiratory failure. Thereafter, he generally improved with supportive care, and was weaned from mechanical ventilation at day At day 50, he returned to the local hospital for rehabilitation. Case 2 A year-old man presented at our emergency department due to pulmonary aspiration while eating.
He had dysphagia, numbness and muscle weakness in the extremities that had started in lower limbs two days before presentation.
His medical history included maintenance hemodialysis for chronic glomerulonephritis, aortic valve replacement for aortic stenosis and pacemaker implantation for sick sinus syndrome at the ages of 67, 70, and 71 years, respectively. A physical examination uncovered decreased breath sounds over the right middle to lower lung fields.
A chest X-ray obtained on the same day revealed mild consolidation in the middle to lower lung area. He was thus diagnosed with aspiration pneumonia and admitted to the intensive care unit day 0. Laboratory findings demonstrated mildly elevated serum CRP 7.
However, muscle weakness in limbs and dysphagia deteriorated Figure 2. Anti-ganglioside antibody was negative, and no other tests were undertaken regarding antecedent infection although he described having diarrhea 30 days before arrival at our hospital.
Thereafter, the muscle weakness and dysphagia probably due to glossopharyngeal and vagus nerve palsy gradually improved, and he was moved to an ordinary ward. However, these symptoms recurred at day 53, and we considered that GBS was the cause. He received IVIg once again, but aspiration pneumonia due to bulbar palsy and muscle weakness deteriorated once again, and he died on day Figure 2 Clinical course of case 2. IVIg, intravenous immunoglobulin.
Discussion We described two patients with GBS and respiratory failure with and without mechanical ventilation. Aspiration pneumonia and asphyxiation are frequently encountered by pulmonologists, but the discrimination of these symptoms associated with GBS is critical. From this viewpoint, Table 1 shows our findings on an intensive review of reports describing the initial onset of GBS associated with antecedent infection Eight of them had underlying pathologies, 13 did not and descriptions were not provided for two.
Notably, none of the Japanese patients with respiratory failure had antecedent EBV infection Table 2. Our data suggested that physicians should search for specific initial symptoms such as upper respiratory tract infection and diarrhea associated with antecedent infection during a diagnostic time frame of 5 to 46 days. The diagnostic duration for our patients was identical to that reported 20 , but to the best of our knowledge, this is the first study to focus on Japanese patients.
Full table Notably some patients had back pain that might have been derived from root inflammation 2 , 8 , 20 , and ascending weakness is the rule, with the lower limbs being initially affected followed by the upper limbs 2 , as in our patients. Furthermore, initially retained reflexes that disappear in patients with established disease 20 also comprises a clinical clue to a diagnosis of GBS.
Relatively symmetrical and bilateral muscle weakness in the limbs progresses over a period of 12 hours to 28 days before reaching a plateau 1 , but botulism and viral myositis can also present with weakness that rapidly progresses over periods of hours to days, and these should be differentiated from GBS.
We found a GBS recurrence rate of 8. Kuitwaard et al. The clinical course of GBS was typical in the patient described herein as Case 1, with obvious changes in tidal volume. Although VC or FVC can be reliable markers for assessing risk for mechanical ventilation, they cannot usually be measured at bedside.
Moreover, peak flow measurements and pulse oximetry are inadequate markers. Thus, our findings emphasize the importance of taking a detailed history to identify even non-specific symptoms that arise within one to four weeks before patients seek medical help.
Conclusions Although our results might not be clinically representative of all patients with GBS, our findings suggested that initial symptoms accompanied by non-specific features such as pyrexia, diarrhea and ascending muscle weakness within the most recent 5 to 46 days could be clinical clues to a diagnosis of GBS even in the setting of respiratory failure. Acknowledgements Disclosure: The authors declare no conflict of interest.
After infra-orbital nerve stimulation, no R1 responses were received bilaterally. Retarded R2 responses were received, while the R2 response of the left side was significantly lower. Electroneurography indicated absence of an f wave when his right median, ulnar and peroneal nerves were stimulated. Absence of an f wave was also observed when his left tibial nerve was stimulated, indicating a mixed demyelinative polyneuropathy with a conduction blockade in multiple nerves and mild axial damage.
Our patient received 40 g intravenous Immunoglobulin for a duration of five days based on clinical suspicion. Our patient received an additional 40 g of intravenous Immunoglobulin a few days later, for a duration of three days, in an attempt to ameliorate the final clinical outcome. Relapse can occur in patients who are treated early in the course of GBS and improve, and brief retreatment with the original therapy is usually effective in these cases [ 5 , 6 ].
The decision was taken to try to ameliorate our patient's neurological condition, which required him to remain resting in bed as any change in position caused inconvenience and a sense of falling.
Intense physiotherapy was applied soon after diagnosis. Our patient markedly improved within two weeks. At that time, while still under anti-CMV treatment, he was able to sit up in bed and on a chair. Later on he was able to stand with support and also progressively take a few steps.
After improvement of his clinical condition, our patient was discharged with appropriate follow-up. Discussion Our patient presented to our emergency department using a wheelchair due to an inability to walk or stand up from a sitting position.
The onset of symptoms was abrupt following an upper respiratory tract infection. As suggested by Kaida et al. Other causes, such as multiple sclerosis MS or other demyelinating syndromes, diabetes mellitus, B12 vitamin deficiency, drugs and chemical neuropathies were excluded following historical, clinical, laboratory and imaging tests.
However, antiviral therapy is not classically recommended in GBS, as the disease is considered post-infectious.
At day 50, he returned to the local hospital for rehabilitation. Our patient markedly improved within two weeks. No abnormalities except degenerative lesions and slightly slipped disks were noted, but these findings were irrelevant to the clinical symptoms. Our data suggested that physicians should search for specific initial symptoms such as upper respiratory tract infection and diarrhea associated with antecedent infection during a diagnostic time frame of 5 to 46 days.
Performing an immunoprecipitation technique or immunoabsorption study using glycolipids or glycoproteins purified from EBV or CMV from patient serum samples would provide evidence for supporting the cross-reactivity between human herpes viruses in the case of our patient, but unfortunately these methods are not available in our hospital. Aspiration pneumonia and asphyxiation are frequently encountered by pulmonologists, but the discrimination of these symptoms associated with GBS is critical. His medical history included type 2 diabetes mellitus, essential hypertension, hyperlipidemia that had been poorly controlled for nine years and obesity.
J Neurol Neurosurg Psychiatry ; His medical history included maintenance hemodialysis for chronic glomerulonephritis, aortic valve replacement for aortic stenosis and pacemaker implantation for sick sinus syndrome at the ages of 67, 70, and 71 years, respectively. In view of the neurological findings, his jugular and lumbar spinal column were scanned. Notably, none of the Japanese patients with respiratory failure had antecedent EBV infection Table 2. Neuromuscular disease causing acute respiratory failure. No To Shinkei ;