Two cases of Guillain-Barré syndrome after cerebral hemorrhage or head trauma

Introduction

Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy that primarily damages human spinal nerve roots and peripheral nerves. One of the possible pathological changes is lymphocyte and macrophage infiltration around the small blood vessels of peripheral nerves, which causes demyelination.^[1] As far as we know, various factors can result in GBS. Almost 2/3 of GBS patients suffer from bacterial or viral infections within 1-4 weeks before onset. Campylobacter jejuni (C. jejuni) is the most common pathogen of the prodromal infection especially in northern China. The most common pathogenesis of GBS is mainly based on hypothetical mechanisms deduced from molecular modeling of prodromal infections, caused by pathogens such as C. jejuni, which can activate the body’s immune system to produce antibodies, resulting in peripheral nerve demyelination or axonal injury. In particular, serum anti-ganglioside antibodies in the acute phase are detectable in approxiately 60% of GBS patients.^[2]

In recent years, weakness of extremities and weakened, or even loss of, tendon reflexes have been reported in some patients with traumatic brain injury, cerebral hemorrhage, spinal cord injury, brachial plexus injury, or pelvic fracture fixation.^[3-6] In this article, we provide detailed clinical data of two patients who were diagnosed with GBS following brain hemorrhage or craniocerebral injury. The diagnosis of our 2 patients is based on the diagnostic criteria of GBS published in 2014, which was modified according to the criteria published in 1990.^[7]

Case report

Discussion

We reported two patients with severe GBS following cerebral hemorrhage or head trauma. In the two cases above, typical clinical manifestations were the sudden occurrence of flaccid tetraplegia, areflexia, hypomyotonia, and respiratory failure, without any sensory dysfunction. NCS of both patients showed decreased CMAP and prolonged distal F wave latencies, indicating motor axon injury, which are common electrophysiological features of AMAN. In addition, the result of the CSF tests indicated albumino-cytologic dissociation, and IVIg proved to be efficient in both patients.

The most common subtypes of GBS are acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and AMAN. In contrast to North America and Europe, where more than 90% of the patients are classified as AIDP, approximately 65% of patients in China suffer from AMAN, a subtype of GBS with only motor fiber damage.^[8] Analogous to our cases, the features of nerve conduction studies demonstrated decreased motor amplitudes with an absence of F waves.

The exact pathogenesis of GBS following brain trauma and hemorrhage is not completely clear. Tan et al.^[6] performed sural nerve biopsy and found phagocytosis of myelin sheath debris in the endoneurium. Tan suggested that head trauma and surgery had elevated serum and CSF myelin basic protein levels, which led to immune system activation to produce anti-myelin antibodies that cause demyelination. Moreover, relevant literature indicates that blood-brain barrier (BBB) damage plays an important role in post-traumatic GBS pathogenesis.^[9,10] Disintegration of the BBB during neurotrauma leads to the accumulation of localized T lymphocytes and macrophages, which may induce the transformation of microglial cells in the nervous system into antigen presenting cells.^[3,11,12] Activated microglia can present post traumatic neuronal debris to the immune system and stimulate B cells to produce antibodies against the myelin sheath, causing demyelination, especially in the peripheral nervous system.^[13] The explanation of GBS following head trauma or hemorrhage may be that some substances originating in the central nervous system, usually being weakly immunogenic, are transported through the disrupted BBB to the peripheral nervous system where they cause demyelination or axonal damage.

It has been reported that anti-ganglioside antibodies are elevated in patients with post traumatic head injury.^[3] Some studies have shown that nearly 80% of AMAN patients are anti-ganglioside antibody positive, especially within the first week of onset.^[4] The best treatment for GBS is IVIg or plasma exchange, both of which aim to remove antibodies. However, serum anti-ganglioside antibodies were negative in case 1. We postulate 2 possible explanations: one is that the antibody level had decreased sharply after 2 courses of IVIg when it was obtained 17 days after onset; the other is the patient suffered from anti-ganglioside antibody negative GBS. The 2 patients reported showed significant recovery due to prompt diagnosis and treatment.

In clinical practice, if a sudden bilateral limb weakness occurs after traumatic injury or hemorrhage, which cannot be explained by findings of imaging and routine laboratory examinations, GBS should be considered.^[14] Early examination of the nervous system, EMG, CSF test, and search for anti-ganglioside antibodies can support the diagnosis of peripheral nerve demyelinating disease. For patients diagnosed with GBS, gamma globulin should be given as soon as possible, if there are no contraindications, otherwise plasma exchange can be used.^[15] Despite the rare reports of GBS following head trauma or brain hemorrhage, we find that it is not as rare as we thought in clinical practice. GBS is not a common disease and it is easy to be ignored or misdiagnosed, especially following other severe situations like trauma and brain hemorrhage. Thus, it is of great significance to enhance the awareness of early diagnosis and early treatment of this special pathogenic GBS, which will increase the survival rate and improve the quality of patients’ life.