A 41-year-old man was admitted to an intensive care unit following respiratory arrest. One day prior to admission, he hadcomplained of nausea and pain involving the lower limbs. On the night of admission, he developed diplopia, dysphagia and rapidly progressivequadriparesis. He developed respiratory failure requiring mechanical lung ventilation 24 hours later. On the fifth day of his hospital stay, the patientbecame comatose with absent brainstem reflexes and appeared to be brain dead. The cerebrospinal fluid showed albuminocytological dissociation. Theelectroencephalogram revealed an alpha rhythmic activity. The electrophysiological evaluation revealed an inexcitability of all nerves. Guillain–Barré syndrome was suspected. With supportive treatment, the patient had a remarkable recovery and now is able to independently conduct his dailyactivities.
Guillain–Barré syndrome, autonomic neuropathy, axonopathy, demyelination, brain death, inexcitablenerves.
Guillain–Barré syndrome (GBS) is an important cause of acute neuromuscular paralysis. Molecular mimicryand a cross-reactive immune response play a crucial part in its pathogenesis, at least in those cases with a previous Campylobacter jejuni infection and with antibodies to gangliosides. The type of previous infection andpatient-related host factors seem to determine the form and severity of the disease. The diagnosis of GBS is based on a combination of clinical andlaboratory features. It is typically a monophasic, sub-acute, symmetrical and predominantly motor neuropathy. In rare cases, GBS can present acutequadriparesis and cranial nerve involvement. We report the observation of a patient who presented a state mimicking cerebral death. In fact, the patient'sefferent nerves were completely dysfunctional and he suffered from fulminant GBS with inexcitable peripheral nerves.
A 41-year-old man was admitted to the intensive care unit (ICU) following respiratory arrest. He hadno previous or concurrent illnesses and was not taking any kind of medication.
There was no history of recent trauma or infection. One day prior to admission,he had complained of lower limb muscular pain and exhaustion. Twelve hours later, he developed diplopia and emesis.
At admission, he had sixth left cranial nerve paralysis, hypophonia, dysphagiaand sensory ataxia. Twelve hours after admission, he was unresponsive and developed gasping respiration. He required immediate intubation and mechanicalventilation due to respiratory arrest. No sedative drugs had been administered.
On examination, he was apyrexial, his heart rate was 85 beats/min and arterial blood pressure was 140/80 mmHg. His pupils were 5 mm wide and did not react tolight. There was no voluntary ocular, facial, tongue or pharyngeal movement. The limbs were flaccid and immobile. Motor power was grade 0 (Medical ResearchCouncil grade) in all four limbs and deep tendon reflexes were absent. On the fifth day of his hospital stay, the patient became comatose with no cephalic orperipheral response to pain and no corneal and gag reflexes. Vestibulo-ocular and oculo-cephalic reflexes were absent. Tests indicated the absence of allbrainstem reflexes and he appeared to be brain dead. Initial and complementary laboratory tests performed, as shown in Table 1, were all normal or negative.
| CBC/Biochemistry | Value | Normal range | Unit |
|---|---|---|---|
| Leucocytes | 10.9 | 4.5-11.0 | 103/μl |
| Erythrocytes | 4.31 | 4.5-6.5 | 106/μl |
| Haemoglobin | 13.4 | 13.0-18.0 | g/dl |
| Platelets | 347.0 | 150.0-450.0 | 103/μl |
| Urea | 43 | 8-53 | mg/dl |
| Creatinine | 0.79 | 0.7-1.2 | mg/dl |
| Sodium | 136 | 136-145 | mEq/l |
| Potassium | 3.9 | 3.5-5.10 | mEq/l |
| Chloride | 98 | 98-111 | mEq/l |
| Calcium | 8.6 | 8.4-10.2 | mg/dl |
| [Q5] Creatinine kinase | 49.0 | <171.0 | U/l |
| AST | 30 | 10-50 | U/l |
| ALT | 48 | 17-63 | U/l |
| C-reactive protein | 5.5 | <6.10 | mg/l |
| CSF examination | Value | Reference | Unit |
| Appearance | Clear | ||
| Cells | <1 | 0-5 | cells |
| Glucose | 67.4 | 40.0- | mg/dl |
| Protein | 92.0 | 15.0- | mg/dl |
| Serologies: blood/CSF | |||
| TORCH | Negative | ||
| Respiratory virus | Negative | ||
| Enterovirus | Negative | ||
| EBV | Negative | ||
| VDRL | Negative | ||
| Borrelia | Negative | ||
| Mycoplasma pneumonia | Negative | ||
| HIV 1, 2 | Negative | ||
| Hepatitis A, B, C | Negative | ||
| H1N1 virus | Negative | ||
| Campylobacter jejuni | Negative | ||
| Antiganglioside antibodies | |||
| GM1, GM1b, GD1a | Negative | ||
| GQ1b, GD3, Gt1a | Negative | ||
| Imagiology exams | |||
| CT scan | Normal | ||
| MRI | Normal | ||
Table 1 - Laboratory tests
An examination of cerebral fluid showed it to have normal pressure, be clear and colourless, and have aprotein concentration of 92 mg/dl (normal <45 mg/dl). It contained 1 mononuclear cell/mm3 and 67.9 mg/dl of glucose (blood glucose was 87 mmol/l). Due to this albuminocytological dissociation, a diagnosis of GBS wassuspected.
CT scan and brain magnetic resonance imaging were normal. Anelectroencephalogram (EEG) carried out on the sixth day of hospitalization revealed posterior alpha activity. Nerve conduction studies and needleelectromyography (EMG) were performed on the eighth hospital day. All the motor and sensory nerves were unexcitable.
The patient was first treated (first 5 days) with IV immunoglobulin, 35 g/day, with no neurologicalresponse. Immediately after, he was treated with six plasma exchanges and repeated IV immunoglobulin (5 more days).
The repeated neurological examinations during the first month are shown in Table 2.
| Day 1 (admission) |
|
| Day 1 (12 hours later) |
|
| Day 2 |
|
| Day 5 |
|
| Day 18 |
|
| Month 2 |
|
| Month 3 |
|
| Month 9 |
|
| Today |
|
Table 2 - Neurologic evolution
A tracheotomy was performed 2 days after admission and weaning from mechanicalventilation was started 2 months later. Three months later, spontaneous ventilation was possiblewithout oxygen. He was able to communicate with staff and relatives. Nowadays, our patient is able to walk unaided on even ground. His hands are stillamyotrophic, and distal proprioception and deep tendon reflexes are still absent. He has stocking-and-glove sensory loss, distal loss of light touch anddysaesthesias. He is physically independent for routine activities.
Fulminant GBS mimicking brain death is a rare occurrence, with about 20 casesreported in the literature[1-4]. As with milder forms, there is a slight male predominance, with peak presentation in the fifth decadeof life, often with a history of a recent minor respiratory or gastrointestinal illness.
Our patient was a 41-year-old man with no history of recent trauma orinfection. The most relevant feature of this case was the initial clinical presentation. He rapidly progressed to fulminant GBS with complete efferentnerve dysfunction resulting in flaccid quadriplegia, total areflexia, absent brainstem reflexes and respiratory paralysis. Hypothermia, metabolic derangements and exposure to drugs or toxins were ruled out. Nevertheless, ourpatient did not meet the criteria for brain death declaration[5], as there was no consistent aetiology, which is an inescapable requirement. Absentresponse with a peripheral nerve stimulator as well as a normal imaging study of the brain and a normal EEG prompted a search for a peripheral cause.Presenting history and rapidly progressive areflexic paralysis, as well as the 'albumin-cytological dissociation' in the cerebrospinal fluid studies,suggested the diagnosis of GBS.
When confronted with a patient in a 'comatose state', the diagnosis of GBS doesnot seem apparent. Pupillary abnormalities have rarely been described[4] and our patient had total ophthalmoplegia, not mentioned in the criteria forthe diagnosis of GBS. Miller-Fisher syndrome, a rare variant of GBS that typically presents with the classic triad of ataxia, areflexia andophthalmoplegia, should always be suspected in such presentations[1]. Fulminant GBS has a poor recovery rate with permanent disabling weakness[2]. The different therapeutic methods are specified in only some cases. It istherefore difficult to establish treatment guidelines for these types of patients. In addition, in a recent study of patients with GBS, including thosewith unexcitable nerves, the outcomes in response to plasma exchange or infusion of gamma globulin, or a combination of both treatments, did not differ[3]. Most patients had a prolonged ICU stay and at the time of discharge they werephysically dependent for routine activities. Death occurred in some cases caused mainly by cardiac arrest related to dysautonomia[4]. Our patient recovered progressively and presently he can carry out routine activitiesindependently. GBS with absent brainstem reflexes is an important variant of GBS to consider, because it is potentially easy to make a misdiagnosis of braindeath with the inherent consequences. This case illustrates the importance of electrophysiological tests and laboratory and imaging studies in patients withsuspected brain death where the cause is not clearly determined.