Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 10535  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
  » Next article
  » Previous article 
  » Table of Contents
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  Article in PDF (38 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

  In this Article
 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  References

 Article Access Statistics
    PDF Downloaded259    
    Comments [Add]    
    Cited by others 4    

Recommend this journal

Year : 2002  |  Volume : 50  |  Issue : 4  |  Page : 528-9

Visual loss with papilledema in Guillain-Barre syndrome.

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012, India.

Correspondence Address:
Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012, India.

  »  Abstract

Papilledema and raised intracranial pressure have been reported in association with Guillain-Barre syndrome. Papilledema is usually asympotomatic or associated with mild visual field defects, without any visual loss. The cerebrospinal fluid protein is usually reported to be high. A case of a 35 year old lady is reported, who presented with headache, diplopia and progressive visual loss in both eyes and limb weakness with hyporeflexia. Optic fundus examination showed bilateral papilledema. She had features of pseudotumor cerebri. Nerve conduction studies were suggestive of polyradiculopathy. The unusual things in this case, were the profound visual loss normal cerebrospinal fluid profiles and the presentation of papilledema before the limb weakness.

How to cite this article:
Kharbanda P S, Prabhakar S, Lal V, Das C P. Visual loss with papilledema in Guillain-Barre syndrome. Neurol India 2002;50:528

How to cite this URL:
Kharbanda P S, Prabhakar S, Lal V, Das C P. Visual loss with papilledema in Guillain-Barre syndrome. Neurol India [serial online] 2002 [cited 2019 Aug 22];50:528. Available from:

   »   Introduction Top

Guillain-Barre syndrome (GBS) or acute inflammatory demyelinating poly neuropathy, usually presents as an areflexic ascending quadriparesis. However atypical and incomplete manifestations are not uncommon. A number of cranial nerves can be involved, facial nerve being the commonest. Papilledema has been reported as a rare complication of GBS. Although blurred discs during the course of GBS were reported earlier, initial reports of definite papilledema appeared in1936.[1] Since then a number of case repors of this entity can be found in literature. Significant among them is a series of four cases by Morley et al.[2] Some initial reports do not have the cerebrospinal fluid (CSF) pressure measurement, but later increased CSF pressure has been reported and these cases were classified as pseudotumor cerebri. Pseudotumor cerebri has also been reported in cases of GBS associated with human immunodeficiency virus infection.[3] Communicating hydrocephalus has also been reported in GBS.[4] Papilledema is usually seen after the limb weakness and is associated with elevated CSF protein. We are aware of only one report of GBS in which pseudotumor cerebri has been documented and the CSF protein was normal.[5] The papilledema usually subsides gradually and visual loss has not been reported before.

   »   Case report Top

A 35 year old lady presented with continuous and bilateral headache for three weeks prior to admission. This was followed by binocular diplopia which was initally present on looking to the left, but after one week, diplopia was present in all directions. At the same time she started having progressive visual impairment in both the eyes. There was no retro orbital pain or pain on moving the eyes. There was total visual loss within one week. One week prior to admission, i.e., two weeks after the onset of symptoms, she developed progressive weakness of all the four limbs, which was symmetrical and was not associated with any radicular pain, wasting or fasiculations. Sensory symptoms were absent. There was no involvement of urinary bladder or bowel. She did not complain of any respiratory distress. On examination she had hypertension (BP-160/110mmHg), and mild tachycardia (pulse-108/min). Central nervous system examination revealed normal higher functions. She had bilateral ptosis, lateral rectus palsy and papilledema. Visual acuity was reduced to just projection of light in both the eyes. Other cranial nerves were normal. Speech was not involved. Motor examination revealed normal tone and bulk of all limbs. No fasiculations or any other abnormal movements were noted. Power was grade 3/5 in proximal and 4/5 to 4+/5 in distal muscles in both upper and lower limbs. Deep tendon jerks were absent and plantars showed flexor response. There was no sensory loss or signs of cerebellar dysfunction. Investigations revealed a normal hemogram and biochemical profile. Cerebrospinal fluid examination revealed raised pressure (420 mm of CSF) and no leukocytes. CSF protein was 20 mg/dl and sugar 76 mg/dl. Gram stain, bacterial culture, VDRL, malignant cytology, cryptococcal antigen, acid-fast bacilli, and flowcytometry were negative in the CSF. Antinuclear antibody, LE cell, anti nuclear cytoplasmic antigen, and rheumatoid factor were negative in blood. Visual evoked responses did not show any wave formation and brainstem auditory evoked responses were normal. CT scan and MRI of the brain were unremarkable. Nerve conduction studies showed absence of `F' waves in upper and lower limbs. A diagnosis of acute inflammatory demyelinating polyradiculopathy with pseudotumor cerebri was made. She was started on treatment with acetazolamide and corticosteroids. Metoprolol was given for the autonomic dysfunction. Her headache slowly subsided. Repeat optic fundus examination showed some features of anterior ischaemic optic neuropathy. This was thought to be secondary to the raised intracranial pressure. She started having a gradual recovery while she was in the hospital.

   »   Discussion Top

The association of papilledema with acute inflammatory demyelinating poly neuropathy has been described in literature. In most of these reports the papilledema appeared after there was an established limb weakness, however, in the present case the papilledema preceded the limb weakness. The CSF proteins were elevated in the cases reported earlier. These elevated proteins were postulated to cause a defect in the proper absorption of CSF at the arachnoid villi, giving rise to raised intacranial pressure.[6],[7] Few cases of hydrocephalus associated with GBS have also been explained on the basis of the same theory. Joynt, however, postulated that the papilledema may be secondary to cerebral edema.[8] Edema of the spinal nerve rootlets seen in GBS has been implicated in causing decreased absorption of proteins at this level and contribute to the raised CSF protein.[4] Pseudotumor cerebri has also been reported with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) but there is only one reported case of raised intracranial pressure in a patient of AIDP, where the CSF protein was normal.[5] Our case had all the features of a pseudotumor cerebri, but with normal CSF proteins. In the earlier reported cases the papilledema was an asymptomatic feature and was detected only during the routine examination of the optic fundus, but in the index case this progressed to a near total visual loss. This prompted us to think that elevated CSF protein may not be the only mechanism for the increased intracranial pressure in patients of Guillain Barre syndrome and this raised intracranial pressure can even lead to visual loss. This also means that patients of GBS, especially the ones who develop papilledema, need to be closely followed up to detect any field defects or visual loss, so that timely intervention can be initiated.


  »   References Top

1.Gilpin SF, Moersch FP, Kernohan JW : Polyneuritis : A clinical and pathologic study of a special group of cases frequently referred to as instances of neuronitis. Arch Neurol Psychiatr 1936; 35: 937-963.   Back to cited text no. 1    
2.Morley JB, Reynolds EH : Papilledema and the Landry -Guillain Barre Syndrome. Brain 1966; 89: 205-222.   Back to cited text no. 2    
3.Gross FJ, Mindel JS : Pseudotumor cerebri and Guillian-Barre Syndrome associated with human immunodeficiency virus infection. Neurology 1991, 41: 1845-1846.   Back to cited text no. 3    
4.Gil Martin RC, Chien LJ : Guillian - Barre Syndrome with hydrocephalus in early infancy. Arch Neurol 1977; 34: 567-569.   Back to cited text no. 4    
5.Sullivan RL, Reeves AG : Normal cerebrospinal fluid protein, increased intracranial pressure and the Guillain -Barre syndrome. Ann Neurol 1977; 1: 108-109.   Back to cited text no. 5    
6.Gardner WJ, Spitler DK, Whitten C : Increased intracranial pressure caused by increased protein content in the CSF : An explanation of papilledema in certain cases of small intracranial and intraspinal tumors and in the Guillain- Barre syndrome. N Engl J Med 1954; 250: 932-36.   Back to cited text no. 6    
7.Feldman S, Landau J, Halpren L : Papilledema in the Guillain - Barre syndrome. Arch Neurol Psychiatr 1955; 73: 678-684.   Back to cited text no. 7    
8.Joynt RJ : Mechanism of production of papilledema in Guillain - Barre syndrome. Neurology 1958; 8: 8-12   Back to cited text no. 8    


Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow