\documentclass[10pt]{article}

\usepackage{fullpage}
\usepackage{setspace}
\usepackage{parskip}
\usepackage{titlesec}
\usepackage[section]{placeins}
\usepackage{xcolor}
\usepackage{breakcites}
\usepackage{lineno}
\usepackage{hyphenat}





\PassOptionsToPackage{hyphens}{url}
\usepackage[colorlinks = true,
            linkcolor = blue,
            urlcolor  = blue,
            citecolor = blue,
            anchorcolor = blue]{hyperref}
\usepackage{etoolbox}
\makeatletter
\patchcmd\@combinedblfloats{\box\@outputbox}{\unvbox\@outputbox}{}{%
  \errmessage{\noexpand\@combinedblfloats could not be patched}%
}%
\makeatother


\usepackage{natbib}




\renewenvironment{abstract}
  {{\bfseries\noindent{\abstractname}\par\nobreak}\footnotesize}
  {\bigskip}

\titlespacing{\section}{0pt}{*3}{*1}
\titlespacing{\subsection}{0pt}{*2}{*0.5}
\titlespacing{\subsubsection}{0pt}{*1.5}{0pt}


\usepackage{authblk}


\usepackage{graphicx}
\usepackage[space]{grffile}
\usepackage{latexsym}
\usepackage{textcomp}
\usepackage{longtable}
\usepackage{tabulary}
\usepackage{booktabs,array,multirow}
\usepackage{amsfonts,amsmath,amssymb}
\providecommand\citet{\cite}
\providecommand\citep{\cite}
\providecommand\citealt{\cite}
% You can conditionalize code for latexml or normal latex using this.
\newif\iflatexml\latexmlfalse
\providecommand{\tightlist}{\setlength{\itemsep}{0pt}\setlength{\parskip}{0pt}}%

\AtBeginDocument{\DeclareGraphicsExtensions{.pdf,.PDF,.eps,.EPS,.png,.PNG,.tif,.TIF,.jpg,.JPG,.jpeg,.JPEG}}

\usepackage[utf8]{inputenc}
\usepackage[ngerman,english]{babel}



\usepackage{float}








\begin{document}

\title{Atypical Guillain--Barre syndrome with T6 sensory level}



\author[1]{Osamah AL-Ameen}%
\author[2]{mohanad  faisal}%
\author[2]{salma mustafa}%
\author[1]{Mohammed alhatou}%
\affil[1]{Hamad Medical Corporation}%
\affil[2]{Hamad medical corportation}%


\vspace{-1em}



  
  \date{June 10, 2024}


\begingroup
\let\center\flushleft
\let\endcenter\endflushleft
\maketitle
\endgroup





\selectlanguage{english}
\begin{abstract}
Guillain-Barr\selectlanguage{ngerman}é syndrome is an acute immune-mediated demyelinating
disease. Typical features include progressive ascending lower extremity
weakness and areflexia. Several variants have been described which can
make the diagnosis challenging . Here, we report a case of GBS
presenting with progressive lower limb weakness and T6 sensory level.%
\end{abstract}\selectlanguage{ngerman}%



\sloppy


\textbf{Introduction:}

Guillain--Barre syndrome (GBS) is an acute immune-mediated inflammatory
demyelinating polyneuropathy, and it is the most common cause of acute
flaccid paralysis worldwide.

It is occurring with an overall incidence of 1 to 2 cases per 100,000
people per year. The disease's incidence rises by about 20\% every
decade of age, and males are more likely to contract it than females
{[}1{]}.

It mainly affects most of the spinal nerve roots and peripheral nerves
and often involves the cranial nerves and is characterized by
symmetrical limb weakness and areflexia {[}2{]}.

Most patients present with an antecedent illness, most commonly an upper
respiratory tract infection or diarrheal illness, before the onset of
progressive motor weakness. Several microorganisms have been associated
with Guillain-Barr\selectlanguage{ngerman}é syndrome, most notably Campylobacter jejuni, Zika
virus, influenza, Mycoplasma pneumoniae, and cytomegalovirus. and in
2020, the severe acute respiratory syndrome coronavirus {[}3{]}.

Diagnostic criteria for GBS were originally proposed for research in
1978 by the National Institute of Neurological Disorders and Stroke
(NINDS). Including, acute progressive and symmetric muscle weakness with
absent or depressed deep tendon reflexes {[}4{]}.

The weakness can vary from mild difficulty with walking to nearly
complete paralysis of all extremities, including facial, respiratory,
and bulbar muscles. Symptoms typically progress over days to four weeks.
Patients may also have mild sensory symptoms and dysautonomia.

Lumbar puncture for CSF analysis should be performed in all patients to
confirm the GBS diagnosis and exclude other sources of the symptoms.
With a typical finding of an elevated CSF protein with a normal white
blood cell count.

Electrodiagnostic studies consisting of nerve conduction studies (NCS)
and electromyography (EMG) are performed in most patients to support the
diagnosis of GBS as well as to provide prognostic information regarding
the nature and severity of nerve dysfunction.

Multiple variants of GBS have been described. Here we will present a
rare case of GBS that presented with lower limb weakness, areflexia, and
atypical sensory level.

\textbf{Case Presentation:}

A 61-year-old Indian female with a past medical history of diabetes
mellitus and chronic mechanical back pain presented with progressively
worsening lower limb weakness, gait difficulty, and reduced sensation in
the abdomen and lower limbs. and numbness in her trunk and bilateral
lower extremities. She denied urinary or bowel incontinence. There were
no abnormal movements or a reduced level of consciousness. Her symptoms
were preceded by a febrile watery diarrheal illness 7 days before the
onset of these symptoms. However, on presentation, her diarrhea had
already subsided. Family and social history were unremarkable. Her vital
signs were within the normal range. Physical~examination showed intact
consciousness, speech, and cranial nerves. Lower limb power was 3/5
proximally and 4/5 distally. Reflexes were 1+ in the upper limb and
areflexia in the lower limb. The sensory exam showed loss of sensation
in all modalities, including light touch, pain, temperature, joint
position, and vibration below the T6 level. Finger to-nose and heel to
shin tests were normal, but gait was unsteady.

The initial workup showed leukocytosis (WBC of 12.000) with a normal
complete metabolic profile. urgent Magnetic resonance images (MRI) of
the whole spine (figure 1) to exclude transverse myelitis or cord
compression were inconclusive apart from mild multilevel degenerative
changes seen in the mid-cervical, mid-dorsal, and lower lumbar regions,
predominantly at the level of C5-C6, C6-C7, T8-T9, L3-L4 and L4-L5. CSF
studies showed protein of 1.57 gm/dl with normal cell counts consistent
with albumin-cytologic dissociation(Table 1). CSF tests for infection
were unremarkable as well, including bacterial and virologic studies.
Nerve conduction studies showed absent F-waves of both perineal and
tibial nerves, otherwise an unremarkable study. Needle examination
revealed positive sharp waves with decreased recruitment in both
iliopsoases, consistent with early neurogenic changes. Denervation was
also seen in the L1-L2 paraspinal muscles.

In view of her clinical presentation of acute flaccid paralysis with
areflexia, unremarkable MRI findings, CSF albumin-cytologic
dissociation, and absent lower extremity F-waves, she was diagnosed with
having possible GBS and she was started on a 5-day course of intravenous
(IV) immunoglobulin with a standard dose of 0.4 gm/kg, which was later
followed by a 5-day course of IV pulse methylprednisolone. At first, her
lower limb weakness worsened to 1/5 and she developed bowel and bladder
dysfunction.

A follow-up MRI of the spine 1 week after the first one did not show any
new changes. Over the next 2 to 4 weeks, the patient started to recover
gradually, with improvement in her sensations first, then full recovery
of motor symptoms with active rehabilitation with the help of an
occupational therapist and physiotherapist. On follow-up four months
later, she remained independent in all activities of daily living.

\textbf{Discussion:}

GBS manifests itself in a variety of ways, ranging from typical
peripheral 4 limb weakness with areflexia to numerous variants that
complicate diagnosis and management. Typical GBS usually presents with
ascending limb weakness with hyporeflexia/areflexia with minor sensory
symptoms and usually involves the 4 upper limbs {[}4{]}. Besides the
typical presentation of GBS, clinical variants are based on the types of
nerve fibers involved (motor, sensory, sensory, and motor, cranial, or
autonomic) and the mode of fiber injury (demyelinating vs axonal) {[}5,
6{]}. The most common mode of nerve fiber injury is demyelinating, while
axonal motor and axonal sensory variants have been described {[}6{]} and
are associated with rapid progression and poor prognosis {[}7{]}. Axonal
variants are usually preceded by campylobacter jejune infection {[}8{]}.

GBS typically has ascending weakness, but descending variants have been
described, including the Miller-Fischer variant, which consists of
ophthalmoplegia, ataxia, and areflexia {[}9{]}. Another cranial variant
is the Bickerstaff brainstem encephalitis, which is characterized by
alteration in consciousness, hyperreflexia, ataxia, and ophthalmoplegia
{[}10{]}.

A less common paraparetic motor variant affects the legs selectively
with areflexia, mimicking an acute spinal cord lesion, and is associated
with back pain {[}11{]}. In most cases, the paraparetic variant is
usually milder compared to the classical GBS, and in 50\% of the cases,
there are abnormalities in upper limb nerves observed in NCS {[}12{]}.

While the sensory symptoms in GBS are typically mild, pure sensory
variants have been described and are usually characterized clinically by
exclusive sensory symptoms and signs that reach their nadir in a maximum
of 6 weeks without related systemic disorders and other diseases or
conditions {[}13{]}.

Our case is a 62-year-old lady who presented with progressive ascending
lower limb weakness with areflexia in addition to the sensory level of
T6. MRI excluded spinal cord compression and demyelination. CSF studies
showed albumin-cytological dissociation. NCS showed absent F-waves of
both the perineal and tibial nerves. She was given intravenous
immunoglobulin followed by five days of IV methylprednisolone, and her
symptoms improved over four weeks. Our most likely diagnosis was GBS
based on the presentation of acute flaccid paralysis, areflexia,
preceding diarrheal illness, CSF studies, and lack of evidence of
demyelination and cord compression on MRI, in addition to the duration
and course of her illness. We think that our case is like the case
reported by Khoo et al. and may represent a new variant of GBS {[}14{]}.
It is important to note that typical NCS findings are not always present
in GBS as it is affected by timing and multiple studies at different
times are needed {[}15{]}.

Another argument is the possibility of transverse myelitis provided that
our patient has sensory level and with the onset of symptoms typical of
transverse myelitis, but the diagnostic criteria {[}16{]} don't apply in
our case as there is an absence of demyelination in repeated MRI and no
CSF leukocytosis.

\textbf{Conclusion:}

GBS varies in its presentation, and atypical presentation with sensory
level may represent a new variant. further studies are needed to confirm
our suspicion.

\textbf{Figures and tables~:}

Table 1: CSF Analysis\selectlanguage{english}
\begin{longtable}[]{@{}lll@{}}
\toprule
\textbf{CSF Analysis} & \textbf{CSF Analysis} & \textbf{Normal
Values}\tabularnewline
Appearance & Clear & Clear\tabularnewline
Color & Colorless & Colorless\tabularnewline
RBC & 333 /uL & (0-2)\tabularnewline
WBC & 8 /uL & (0-5)\tabularnewline
CSF Glucose & 7.49 mmol/L & (2.22-3.89)\tabularnewline
CSF Protein & 1.57 gm/L & (0.15-0.45)\tabularnewline
Albumin Cytologic Dissociation & Present & Absent\tabularnewline
CSF Culture & Negative &\tabularnewline
\bottomrule
\end{longtable}

Figure 1: MRI of the thoracic spine\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

References:

1. Yuki N, Hartung HP. Guillain-Barr\selectlanguage{ngerman}é syndrome. N Engl J Med.
2012;366(24):2294-304. doi: 10.1056/NEJMra1114525. PubMed PMID:
22694000.

2. Criteria for diagnosis of Guillain-Barré syndrome. Ann Neurol.
1978;3(6):565-6. doi: 10.1002/ana.410030628. PubMed PMID: 677829.

3. Shahrizaila N, Lehmann HC, Kuwabara S. Guillain-Barré syndrome.
Lancet. 2021;397(10280):1214-28. Epub 20210226. doi:
10.1016/s0140-6736(21)00517-1. PubMed PMID: 33647239.

4. Dimachkie MM, Barohn RJ. Guillain-Barré syndrome and variants. Neurol
Clin. 2013;31(2):491-510. Epub 20130219. doi: 10.1016/j.ncl.2013.01.005.
PubMed PMID: 23642721; PubMed Central PMCID: PMC3939842.

5. Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, et
al. An acute axonal form of Guillain-Barré polyneuropathy. Brain.
1986;109 ( Pt 6):1115-26. doi: 10.1093/brain/109.6.1115. PubMed PMID:
3790970.

6. Griffin JW, Li CY, Ho TW, Tian M, Gao CY, Xue P, et al. Pathology of
the motor-sensory axonal Guillain-Barré syndrome. Ann Neurol.
1996;39(1):17-28. doi: 10.1002/ana.410390105. PubMed PMID: 8572662.

7. Hiraga A, Mori M, Ogawara K, Hattori T, Kuwabara S. Differences in
patterns of progression in demyelinating and axonal Guillain-Barré
syndromes. Neurology. 2003;61(4):471-4. doi:
10.1212/01.wnl.0000081231.08914.a1. PubMed PMID: 12939419.

8. Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW, et al.
Guillain-Barré syndrome in northern China. Relationship to Campylobacter
jejuni infection and anti-glycolipid antibodies. Brain. 1995;118 ( Pt
3):597-605. doi: 10.1093/brain/118.3.597. PubMed PMID: 7600081.

9. FISHER M. An unusual variant of acute idiopathic polyneuritis
(syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med.
1956;255(2):57-65. doi: 10.1056/NEJM195607122550201. PubMed PMID:
13334797.

10. BICKERSTAFF ER, CLOAKE PC. Mesencephalitis and rhombencephalitis. Br
Med J. 1951;2(4723):77-81. doi: 10.1136/bmj.2.4723.77. PubMed PMID:
14848512; PubMed Central PMCID: PMC2069534.

11. Ropper AH. Unusual clinical variants and signs in Guillain-Barré
syndrome. Arch Neurol. 1986;43(11):1150-2. doi:
10.1001/archneur.1986.00520110044012. PubMed PMID: 2946281.

12. van den Berg B, Fokke C, Drenthen J, van Doorn PA, Jacobs BC.
Paraparetic Guillain-Barré syndrome. Neurology. 2014;82(22):1984-9. Epub
20140507. doi: 10.1212/WNL.0000000000000481. PubMed PMID: 24808021.

13. Uncini A, Yuki N. Sensory Guillain-Barré syndrome and related
disorders: an attempt at systematization. Muscle Nerve.
2012;45(4):464-70. doi: 10.1002/mus.22298. PubMed PMID: 22431077.

14. Khoo CS, Ali AH, Remli R, Tan HJ. A case of Guillain-Barré syndrome
(GBS) presenting with acute urinary retention and T6 sensory level. Clin
Med (Lond). 2018;18(4):308-10. doi: 10.7861/clinmedicine.18-4-308.
PubMed PMID: 30072555; PubMed Central PMCID: PMC6334053.

15. Rath J, Schober B, Zulehner G, Grisold A, Krenn M, Cetin H, et al.
Nerve conduction studies in Guillain-Barré syndrome: Influence of timing
and value of repeated measurements. J Neurol Sci. 2021;420:117267. Epub
20201213. doi: 10.1016/j.jns.2020.117267. PubMed PMID: 33352506.

16. Group TMCW. Proposed diagnostic criteria and nosology of acute
transverse myelitis. Neurology. 2002;59(4):499-505. doi:
10.1212/wnl.59.4.499. PubMed PMID: 12236201.

\textbf{Acknowledgment:}

Open access article was funded by Qatar National library

\textbf{Availability of data:}

The data that support the findings of this study are available from the
corresponding author upon reasonable request.

\textbf{Conflict of interest:}

The authors have no conflict of interest to declare.

\selectlanguage{english}
\FloatBarrier
\end{document}

