Pharyngo-Cervico-Brachial Variant of Guillain-Barré or Miller Fisher Syndromes? When the Overlap Is Misleading

Round 1

Reviewer 1 Report

In this manuscript the authors reported the case of a 74-year-old patient with acute onset of right ptosis, horizontal diplopia, dysphonia with nasal voice, and difficulty swallowing. The authors reported that clinical examination at admission showed a complete bilateral ophthalmoplegia associated with a bilateral ptosis predominant on the right, a static ataxia not increased by the closure of the eyes, a diffuse osteotendinous areflexia, and vibration sense was decreased in the four extremities. CSF analysis showed cytoalbuminologic dissociation. Interestingly, the areflexic patient had a normal electrophysiologic study in the four limbs and in the facial nerve. Moreover, antiGQ1B antibodies were negatives, whereas antiGT1a were positives. I agree with the authors that the reported patient was affected by an overlapping syndrome with a polyneuritis cranialis variant of GBS, instead of a classic form of GBS or MFS. Thus, the title of this case report should be modified accordingly. In addition, the authors should comment: 1) the unexpected results coming from the neurophysiological examination; 2) the decision to not treat a patient with bulbar symptoms at admission.

Author Response

I would like first of all to thank you for your valuable comments. Indeed, it did help to improve the quality of our manuscript.

Please find below the response to your comments.

I agree with the authors that the reported patient was affected by an overlapping syndrome with a polyneuritis cranialis variant of GBS, instead of a classic form of GBS or MFS. Thus, the title of this case report should be modified accordingly.

We appreciate your suggestion, it was taken into consideration.

The title was modified into:

Pharyngo-cervico-brachial variant of Guillain-Barré or Miller Fisher syndromes? when the overlap is misleading.

 In addition, the authors should comment:

 1) the unexpected results coming from the neurophysiological examination;

We did add a comment regarding this point in the text:

In the observation as:

A large electroneuromyographic examination was realized on day 6 including sensory and motor nerve studies, F waves, H reflex, and repetitive nerve stimulation in the four limbs and in the facial nerve. Surprisingly, the outcome was symmetrically normal.

In the discussion as:

Our patient presented with rich clinical signs, however the EMG/NCS didn’t reveal any abnormality. This result wasn’t expected and increased the doubts about an overlap between a MFS and an incomplete form of PCB variant of GBS.  A literature review found that NCS findings in PCB/FS have been rarely and variably reported.  Similar to our patient, the initial NCS could not detect any abnormalities but serial electrophysiological studies may be valuable.

 2) the decision to not treat a patient with bulbar symptoms at admission.

Thank you so much for your remark.

Actually, this point wasn’t clear in the description of the disease course. The immunotherapy was proposed to the patient but unfortunately he couldn’t afford it.

It was added to the text as follow:

In view of the clinical presentation, we proposed an intravenous immunoglobulins (IVIG) course, but the treatment was not affordable for the patient.  

Reviewer 2 Report

This is an additional report on the spectrum of variants of the GBS.  The main feature seem to be elements of the Miller Fisher syndrome MLF, and the point of this paper is  the additional of other (caudal) cranial nerves. Being a combination of main features belonging to theMiller Fisher and some elements of the Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry 2014 Mar;85(3):339-44. doi: 10.1136/jnnp-2013-305397. 

The usual discussion of the MLF also contains considerations of central parenchymal lesions, which needs to be added.

The structure is good. Although english is good  even very good, some grammatical and spelling errors can be found in many places. Please ask a native speakers to go over the final version.

Author Response

We thank you for your remarks and comments and also the link to the article you shared with us. All of it helped to improve our manuscript.

1. The usual discussion of the MLF also contains considerations of central parenchymal lesions, which needs to be added.

We added comments about the central parenchymal lesions. In the discussion part of the article we added:

To further support our diagnosis, brain MRI was done and did not show any brainstem abnormalities ruling out an overlap with Bickerstaff rhomboencephalitis or structural lesions that can mimic the patient’s presentation. Hence, MRI is the most early important paraclinical investigation. It should be normal in the case of MFS and PCB variant of GBS, although there may be gadolinium enhancement of proximal cervical nerve roots

2. The structure is good. Although english is good  even very good, some grammatical and spelling errors can be found in many places. Please ask a native speakers to go over the final version.

Thank you for your positive comments.

Round 2

Reviewer 1 Report

The authors satisfied my suggestions.