Guillain Barre Syndrome: A Rare Presentation Mimicking Acute Stroke

Guillain Barre Syndrome (GBS) is an acute neuromuscular weakness and paralysis associated with areflexia and often spontaneous recovery, but carries the potential risk of respiratory depression owing to muscle weakness. Worldwide, 1 to 3 cases/100,000 are reported. The syndrome is most commonly reported as symmetrical ascending weakness in arms and legs accompanied by hyporeflexia or areflexia. Sensory disturbances are not required for diagnosis, but may or may not be present. Acute inflammatory demyelinating poly-radiculoneuropathy (AIDP) is the most common variant, but acute motor and sensory axonal neuropathy (ASMAN) is more severe and usually leads to partial or slow recovery. We present a case of GBS presenting with asymmetric weakness and sensory disturbance in a patient with bloody diarrhea of unknown etiology. This patient had asymmetrical paralysis mimicking stroke, but the physical findings, laboratory studies, normal CT and MRI of the brain, Electromyogram (EMG) and the patient’s improvement with Intravenous Immunoglobulin (IVIG) support the diagnosis of GBS. People with inflammatory bowel disease are at increased risk of Case Study British Journal of Medicine & Medical Research, 4(15): 3009-3015, 2014 3010 developing GBS. Persons with antecedent Campylobacter jejuni infections are 77 percent more likely to contract GBS compared to the general population, and Cytomegalovirus (CMV) and Epstein Barr virus (EBV) are also implicated risk factors.

developing GBS. Persons with antecedent Campylobacter jejuni infections are 77 percent more likely to contract GBS compared to the general population, and Cytomegalovirus (CMV) and Epstein Barr virus (EBV) are also implicated risk factors.

CASE PRESENTATION
49 years old male with past medical history of gastritis, ulcerative colitis and Deep Vein Thrombosis (DVT) presented to Emergency Department (ED) with severe crampy lower abdominal pain and bloody diarrhea for 7 days. The patient was in no acute distress and he was afebrile, and had respiratory rate 20 breaths/min, heart rate 97/minutes, blood pressure 100/75mmHg and O2 saturation of 97% on ambient air. He had tenderness in the left lower quadrant (LLQ), and focused neurological examination was unremarkable. Laboratory studies sowed hemoglobin 10.5 g/dl, hematocrit 31.6%, WBC 7700/uL and Platelet 396,000/uL. The patient was admitted to the regular floor for treatment with prednisone, mesalamine and metronidazole for ulcerative colitis flare up.
Next day, he almost fell on his way to the bathroom due to left leg weakness. On further questioning, it was discovered that he began to have this progressively worsening weakness 3 weeks ago. Detailed neurological examination revealed that muscle strength was 2/5 on the left side and 4/5 on the right. All the deep tendon reflexes were absent Sensory sensations were impaired more on the left side than right side. Cranial nerves were intact and there were no cerebellar signs. Given the asymmetry of neurological symptoms, the possibility of acute stroke was considered. However, CT of the brain without contrast found no evidence of intracranial hemorrhage or acute stroke. A brain MRI without contrast showed no evidence of infarction or any other abnormality.
MRI lumbar spine revealed mild degenerative findings with a small left L5-S1 foraminal disk herniation. MRI of cervical spine showed mild degenerative findings with no significant stenosis and no cervical cord compression. EMG and nerve conduction study (NCS) of both lower extremities revealed bilateral poly-neural peripheral demyelinating disease with slowed conduction time highly suggestive of a demyelinating disorder most likely GBS.
On 6 th day of admission, the patient began complaining of difficulty in breathing. The patient was transferred to the Intensive Care Unit (ICU) for GBS with impending respiratory failure, due to vital capacity (VC) of 1.010Liter and a negative inspiratory force (NIF) of -12cm H2O. Orthostatic hypotension was also noted. An EMG and NCS found the left leg to have slowed conduction time suggestive of a demyelinating disorder most likely GBS. There was no albuminocytological dissociation on CSF analysis done on day 11 and 17 (Table 1).

DISCUSSION
Differential diagnosis of asymmetric weakness and sensory disturbance includes but not limited to Amyotrophic lateral sclerosis (ALS), Mononeuritis multiplex (MNM), multiple sclerosis (MS), Acute stroke and Guillian-Barre syndrome.
People with inflammatory bowel disease are at increased risk of developing GBS [1].
The incidence is less than 3 per 100,000 persons, with a higher incidence in males and increasing risk with aging [2,3]. Persons with antecedent Campylobacter jejuni infections are 77 percent more likely to contract GBS compared to the general population, and EBV and CMV infection are also implicated risk factors [4,5,6]. Our patient had history of IBD and presented with bloody diarrhea, and hence had the risk factors for developing GBS.
ALS can involve both the upper and lower motor neurons. Patient will have symptoms of muscle atrophy and or wasting, dysphasia, and skeletal muscles cramping and twitching. Typically ALS is common in elderly male. There is no specific test for diagnosis of ALS, and this is a clinical diagnosis of exclusion. Patients will have normal blood tests, nerve conduction studies, MRIs and CSF analysis.
The patient will present with a slow onset of combination of the UMN and LMN symptoms [7]. Characteristically ALS does not exhibit any sensory disturbances [8].
Mono-neuritis multiplex (MNM) is characterized as an asymmetrical progressive peripheral neuropathy in two or more nerves in separate parts of the body that is most common secondary to vasculitis. Serology markers including raised ESR, Rheumatoid Factor, and p-ANCA can be positive. This patient had sub-acute neurological deterioration and serum markers of raised ESR, Rheumatoid Factor, and p-ANCA were negative. Multiple sclerosis and acute stroke were excluded by negative brain MRI.
GBS is most commonly reported as symmetrical weakness starting in the legs [9]. The National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) formulated a diagnostic criterion that addresses 85 percent of all GBS cases [10]. The diagnostic criteria for the syndrome include progressive weakness proximally and distally in more than 2 limbs for less than 4 weeks and areflexia [11]. Supportive signs and symptoms to diagnose GBS include sensory loss, elevation of sedimentation rate, hyponatremia secondary to inappropriate antidiuretic hormone release, and decrease in neuromuscular conduction, and normal cell count in CSF with elevated protein [11,12].
Respiratory failure secondary to respiratory muscle weakness occurs in 9 percent of patients [13]. Paresthesia in hands and feet occur in greater than 80 percent of patients, but sensory impairment is usually mild. Autonomic dysfunction can occur in 70 percent of patients; common manifestations are orthostatic hypotension, loss of sweating, urinary retention and ileus [14].
The most common form of GBS in the United States is acute inflammatory demyelinating poly-radiculoneuropathy (AIDP) accounting for85 to 90 percent of the cases. AIDP classically presents with progressive symmetrical ascending paralysis and with areflexia. The mechanism is thought to be an autoimmune attack targeting nerve roots leading to demyelination.
A more severe form of GBS is acute motor and sensory axonal neuropathy (ASMAN). AMSAN has the same criteria as AIDP, but extends to sensory fibers in addition to motor fibers. It is secondary to axonal degeneration, which commonly leads to either a slower recovery or partial recovery [15].
The GBS is commonly preceded by a Campylobacter jejuni infection, but can also be associated with Cytomegalovirus and Epstein Bar virus [16]. Campylobacter jejuni infections are commonly characterized by low-grade fever, abdominal cramps and 8-10 watery or bloody bowel movements daily [17]. CMV most commonly presents with diarrhea, fever, gastrointestinal bleeding and abdominal pain in immunocompromised patients or those with inflammatory bowel disease [18].
Treatment options include is intravenous immunoglobulin and plasma exchange [19]. Clinicians must monitor vital capacity and respiratory rate to predict respiratory failure, and blood pressure and heart rate to monitor autonomic dysfunction.
The difference between our patient and other documented cases was that our patient had profound unilateral motor and sensory deficits along with unilateral loss of sweating. However, the patient fits the GBS criteria; he had a loss of motor function in 2 limbs with areflexia. The patient also had many of the supporting criteria; he had sensory loss and the EMG studied revealed demyelination of peripheral nerves.
Recognition of acute stroke involves taking careful clinical history of abrupt onset of neurological deficit, and focal neurological signs might include unilateral weakness or numbness of face, arm, or leg, visual field defect or speech disturbance [20].
Reports of asymmetrical GBS exist but to our knowledge data does not exist discussing the frequency of asymmetrical presentation. We propose our patient developed atypical GBS. The patient did not present with symmetrical ascending paralysis, but the physical findings, laboratory studies, the EMG and the patient's improvement with IVIG support the diagnosis of GBS.

CONCLUSION
GBS, on rare occasions, can present with asymmetrical weakness and sensory disturbance mimicking acute stroke. People with inflammatory bowel disease are at higher risk of developing GBS. High index of suspicion is required for clinical diagnosis and standard tests including EMG help establish the diagnosis. Intensive monitoring for respiratory failure and autonomic dysfunction is very imperative. Standard treatment with IVIG or plasma exchange is the mainstay of therapeutic intervention.

CONSENT
Not applicable.