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Table of Contents    
CASE REPORT
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1397-1399

A Hypopituitarism Patient with Bickerstaff's Brainstem Encephalitis Overlapped by Guillain-Barré Syndrome: A Case Report


1 First Clinical Medical School; Neurology Department, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China
2 Neurology Department, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China

Date of Submission23-Jan-2018
Date of Decision01-Apr-2018
Date of Acceptance12-Dec-2019
Date of Web Publication30-Oct-2021

Correspondence Address:
Zhi-Bing Wu
Neurology Department, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, 16 JiChang Rd, Guangzhou - 510 405
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.329589

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 » Abstract 


We present a rare case of Bickerstaff's brainstem encephalitis (BBE) overlapped with Guillain-Barré syndrome (GBS), which might be triggered by the patient's hypopituitarism condition. A 36-year-old woman with a history of hypopituitarism presented with a headache on the first day. Gradually, diplopia, ataxia, dysarthria, dysphagia, hypoesthesia, limb weakness, hypersomnolence, and respiratory muscle paralysis were developed in less than ten days. Based on brain computed tomography scan, magnetic resonance imaging scan, nerve conduction studies, cerebrospinal fluids analysis, anti-ganglioside antibodies and hormones tests, and clinical investigations, we diagnosed the patient with BBE overlapped with GBS. Treatment with corticosteroids and immunoglobulin resulted in clinical improvement. To our knowledge, this is the first case report of a hypopituitarism patient with BBE overlapped by GBS in English literature. Hypopituitarism patients have immune dysfunction. Based on previously reported autoimmune diseases associated with triggering GBS and its subtypes, hypopituitarism could be considered a noninfectious trigger.


Keywords: Anti-ganglioside antibodies, autoimmune disease, Bickerstaff's brainstem encephalitis, Guillain-Barré syndrome, hypopituitarism, Sheehan's syndrome
Key Message: As an autoimmune disease that can cause humoral and cellular immune dysfunction, hypopituitarism could be a noninfectious trigger for GBS and its' subtypes.


How to cite this article:
Deng CX, Wu ZB, Yu ZM. A Hypopituitarism Patient with Bickerstaff's Brainstem Encephalitis Overlapped by Guillain-Barré Syndrome: A Case Report. Neurol India 2021;69:1397-9

How to cite this URL:
Deng CX, Wu ZB, Yu ZM. A Hypopituitarism Patient with Bickerstaff's Brainstem Encephalitis Overlapped by Guillain-Barré Syndrome: A Case Report. Neurol India [serial online] 2021 [cited 2021 Dec 2];69:1397-9. Available from: https://www.neurologyindia.com/text.asp?2021/69/5/1397/329589




Guillain-Barré syndrome (GBS) defines the spectrum of related autoimmune-mediated acute inflammatory neuropathies, and Bickerstaff's brainstem encephalitis (BBE) is one of it.[1] BBE's immunopathological pattern involves both the peripheral nervous system (PNS) and the central nervous system (CNS).[2],[3] A case presenting the three core features of BBE (hypersomnolence, ophthalmoplegia, and ataxia) with limb weakness is diagnosed as BBE/GBS overlap.[1],[4] The immunopathological mechanisms of GBS and its subtypes can be triggered by infections, autoimmune diseases, and immunocompromised states.[5]


 » Case History Top


A 36-year-old woman with a five-year history of Sheehan's syndrome was admitted to our hospital presenting with a four-day history of a severe headache, malaise, anorexia, unsteady gait with diplopia, and a one-day history of fever (38.7°C) with projectile vomiting. She had herself discontinued medications for hypopituitarism of prednisolone and L-thyroxine several weeks before admission.

On admission, she was alert and oriented. Physical examination revealed bilateral decreased pupil light reflexes, abduction palsy, mild rigidity in nuchal, and hyporeflexia of all limbs with bilateral Babinski's sign. Brain computed tomography scan suspected generalized cerebral edema [Figure 1]a. Laboratory tests revealed hyponatremia and anterior pituitary hormone deficiency including decreased cortisol, thyroid-stimulating hormone, free triiodothyronine, free thyroxine, follicle-stimulating hormone, luteinizing hormone, prolactin, progesterone, and testosterone. The cerebrospinal fluids (CSF) showed an increased leukocyte count of 55 cells/μL (71.0% mononuclear cells) and opening pressure (220 mm H2O), with normal levels of protein, chloride, glucose, and no oligoclonal bands. Cultures and bacteriological tests of blood and CSF, and total blood count were negative. With a preliminary diagnosis of hypopituitarism with hyponatremic encephalopathy, pulse therapy of methylprednisolone (500 mg per day) was initiated. In the first 16 h, the serum sodium level was elevated approximately 0.325 mmol/(L·H) via intravenous fluid therapy.
Figure 1: Patient's brain CT and MRI scan. (a) CT (axial) showed swelling in the white matter with smaller lateral ventricles. (b) MRI (sagittal T1WI) showed the pituitary gland was thin, flattened, and displaced against the backside of the sellar floor (arrow). Noted the absence of a posterior pituitary “bright spot”

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After the initial treatment, her headache and fever resolved. However, she further developed hypersomnolence, dysarthria, dysphagia, bilateral ptosis, bilateral ophthalmoplegia, absent oculocephalic reflexes, hypoesthesia of the glove-and-stocking type, limb weakness in all extremities, ataxia, and urine retention. On hospital day four she developed respiratory muscle paralysis and was then placed on mechanical ventilation. By that time, the serum sodium, glucose, creatinine, urea, and ammonia were tested negative. Brain magnetic resonance imaging [Figure 1]b examined at hospital days three and 19 both showed an empty sella with an atrophied pituitary. With and without gadolinium enhancement, no plaques in the brainstem or other parts of the brain were detected. The serum anti-GQ1b IgG antibodies were positive. IgG antibodies to GQ1b, GM1, GM2, GD1a, GD1b, and GT1b were positive in CSF. The nerve conduction studies showed reduced amplitudes with decreased conduction velocities in the right ulnar and median sensory nerves. Bilateral tibial motor nerves showed prolonged distal latencies. F-wave responses were normal. BBE/GBS overlap was diagnosed and administration of immunoglobulin (0.4 g/kg per day) for five-day was initiated. After a clinical plateau, her condition improved gradually, and complete remission was achieved 6 months after the onset.


 » Discussion Top


Symptoms of hypopituitarism depend on the degree of hormone deficiencies. Commonly occurring in hypopituitarism patients, hyponatremia can lead to hyponatremic encephalopathy which can cause neurological symptoms. However, hyponatremic encephalopathy was ruled out since our patient's mental status continued to deteriorate post-correction of the serum sodium level and resolution of intracranial hypertension. Moreover, lacking clinical evidence ruled out central pontine myelinolysis. Diagnosis of BBE/GBS overlap was made according to the three core clinical features of BBE with motor peripheral neuropathies and was supported by the presence of anti-GQ1b antibody. Considering the presence of albumin-cytological dissociation as highly time-dependent, the absence of it did not negate our diagnosis.

Antecedent infections are common in BBE.[2],[5] Tan et al. gathered 38 cases of GBS mimicking brain death (possible BBE/GBS overlap cases) and antecedent infections were reported in 24 cases.[6] Our patient had a fever on admission. However, the timeline of her possible infection to the onset of the neuropathies was inconsistent with the reported timeline of an infectious trigger to the onset of GBS.[5] The pathological mechanisms underlying autoimmune diseases triggering GBS and its subtypes are unclear. Hypopituitarism patients are more susceptible to infection and likely to develop autoimmune diseases because of humoral and cellular immune dysfunctions.[7] Previously, a case of a hypopituitarism patient who insidiously developed cognitive impairment, cerebellar ataxia, and demyelinating neuropathy was reported; the patient recovered after hormone replacement, suggesting a causal relationship between the hypopituitarism condition and the neuropathies.[8] In Chinese literature, a pan-hypopituitarism patient with acute areflexic paralysis, albumin-cytological dissociation, and no antecedent infection was diagnosed with GBS; the possible trigger of GBS was linked to the ill-managed hypopituitarism condition.[9] Given the above information, it is reasonable to suggest that our patient's hypopituitarism condition might have played a role in the pathological mechanism in BBE/GBS overlap.

The antibodies produced in autoimmune diseases could include those responses against components of myelin basic protein and neuritogenic protein, thereby causing polyneuropathies.[10] Anti-ganglioside antibodies (AGAs) are suggested to be produced peripherally and penetrate the CNS through the blood-brain barrier.[3] However, the diverse kinds of AGAs in our patient's serum and CSF cannot be explained by transudation from the circulation to the CNS. Therefore, different types of mechanisms might have taken place in the PNS and CNS.

Prognosis of BBE with and without limb weakness is overall good with complete remission or minor residuals.[4] However, poor prognoses were indicated in the 28 cases of GBS mimicking brain death reported by Tan et al. (2 deaths and 11 cases with disabling residuals); although these cases are evidently more severe.[6] Undoubtedly, failure to promptly recognize this variant, especially when coma with loss of brainstem reflexes was presented, could lead to adverse outcomes.

Based on this case, the following questions are raised that require further investigation. (1) Whether the varied kinds of AGAs in the serum and CSF can be interpreted as the existence of different types of immunopathological mechanisms in the PNS and CNS. (2) Whether hypopituitarism can be a noninfectious trigger for GBS and its subtypes.

Acknowledgments

We received funding from the State Administration of Traditional Chinese Medicine of the People's Republic of China (No. 211020030304).

Financial support and sponsorship

We received funding from the State Administration of Traditional Chinese Medicine of the People's Republic of China (No. 211020030304).

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Wakerley BR, Uncini A, Yuki N. Guillain-Barre and Miller Fisher syndromes-new diagnostic classification. Nat Rev Neurol 2014;10:537-44.  Back to cited text no. 1
    
2.
Odaka M, Yuki N, Yamada M, Koga M, Takemi T, Hirata K, et al. Bickerstaff's brainstem encephalitis: Clinical features of 62 cases and a subgroup associated with Guillain-Barre syndrome. Brain 2003;126:2279-90.  Back to cited text no. 2
    
3.
Ito M, Kuwabara S, Odaka M, Misawa S, Koga M, Hirata K, et al. Bickerstaff's brainstem encephalitis and fisher syndrome form a continuous spectrum: clinical analysis of 581 cases. J Neurol 2008;255:674-82.  Back to cited text no. 3
    
4.
Dimachkie MM, Barohn RJ. Guillain-Barre syndrome and variants. Neurol Clin 2013;31:491-510.  Back to cited text no. 4
    
5.
Wakerley BR, Yuki N. Infectious and noninfectious triggers in Guillain-Barre syndrome. Expert Rev Clin Immunol 2013;9:627-39.  Back to cited text no. 5
    
6.
Tan CY, Ahmad SB, Goh KJ, Latif LA, Shahrizaila N. Overlap of Bickerstaff brainstem encephalitis/Guillain-Barre syndrome simulating brain death. Neurol India 2018;66:1475-80.  Back to cited text no. 6
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7.
Mukherjee A, Helbert M, Davis J, Shalet S. Immune function in hypopituitarism: Time to reconsider? Clin Endocrinol (Oxf) 2010;73:425-31.  Back to cited text no. 7
    
8.
Poujois A, Antoine J, Mosnier J, Thomas-Antréion C, Mounier C, Michel D. Multiple neurological complications in a case of anterior pituitary insufficiency. Rev Neurol (Paris) 2004;160:354-6.  Back to cited text no. 8
    
9.
Ye X, Xue Q, Du H, Wang Y, Wang J. A Case Report of Panhypopituitarism with Guillain-Barré Syndrome (originally in Chinese). Jiangsu Med J 2011;37:1482-3.  Back to cited text no. 9
    
10.
van Laarhoven H, Rooyer F, van Engelen B, van Dalen R, Berden J. Guillain-Barré syndrome as presenting feature in a patient with lupus nephritis, with complete resolution after cyclophosphamide treatment. Nephrol Dial Transplant 2001;16:840-2.  Back to cited text no. 10
    


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