Unusual clinical presentation of Guillain Barre syndrome: a

case report

Presentación clínica aguda inusual del síndrome de Guillain

Barre: A propósito de un caso

Submitted 13 Jun 2018

Accepted 19 Jun 2018

Published 06 Dec 2018

Editor in chief

Isaac Kuzmar

editor@revcis.com

Academic editor

Vladimir Ospino

Corresponding author

Montaño - Lozada, JM,

Juanma2499@hotmail.com

DOI 10.17081/innosa.60

2018 - et al. Distributed

under

Creative Commons CC-BY

4.0

Montaño - Lozada, JM

,Licona, Erick.

,Marenco Gómez, Aristides

,Espejo - Zapata, LM

Montoya-Jaramillo Mario, Herrera, Felipe

Universidad del Sinú, Cartagena, Colombia,

Universidad Metropolitana, Barranquilla, Colombia.,

Unidad Central del Valle del Cauca, Tuluá, Colombia.,

Clínica Cartagena del mar, Cartagena, Colombia

ABSTRACT

Background: Guillen barre syndrome is the most frequent cause of flaccid paralysis in the

world, it is characterized by an acute demyelinating, autoimmune and multiple etiology

polyneuropathy, among which are included infectious agents such as Campylobacter

jejuni, Zika virus, of genetic and environmental factors.

Case Report: We present the case of a 56-year-old Colombian male patient with a history

of hypertension, who entered the intensive care unit with symptoms of atypical asymmetric

motor neurological compromise, which rapidly progressed to ventilatory failure and

subsequent confinement síndrome.

Discussion: Guillen barre síndrome establishes a potentially fatal disease, the semiology

of pain, paresthesia, symmetric-progressive, distal weakness, instability, hipo/areflexia,

constitute a neurological emergency. There are Clinical variants establishing a great.

RESUMEN

Introducción: El síndrome de guillan barré es la causa más frecuente de parálisis flácida

en todo el mundo, se caracteriza por ser una polineuropatía aguda desmielinizante,

autoinmune y de etiología múltiple, entre los que se incluyen agentes infecciosos como el

campilobacter jejuni, virus del zika, además de factores genéticos y ambientales.

Caso clínico Se presenta el caso de un paciente masculino de 56 años, colombiano, con

antecedentes de hipertensión arterial, quien ingresa a la unidad de cuidados intensivos

con síntomas de compromiso neurológico motor asimétrico atípico, el cual progresó

rápidamente a falla ventilatoria y posterior síndrome de enclaustramiento.

Discusión: El síndrome de guillan barré establece una enfermedad potencialmente mortal,

la semiología de dolor, parestesia, debilidad distal simétrica-progresiva, inestabilidad e

hipo/arreflexia, constituyen una emergencia neurológica. Existen variantes clínicas

estableciendo un gran reto diagnóstico, teniendo en cuenta que el tratamiento oportuno

podría tener relación directa con el pronóstico de la enfermedad.

Keywords Guillain-Barre Syndrome; Inflammatory Demyelinating Polyradiculoneuropathy,;

Acute Inflammatory Polyneuropathies

Palabras clave Síndrome de Guillain-Barré; Polirradiculoneuropatía Desmielinizante

Inflamator; Polineuropatía Inflamatoria Aguda

How to cite this article: Montaño - Lozada, JM, Licona. Erick, Marenco Gómez, Aristides, Espejo - Zapata, LM, Montoya-Jaramillo Mario, Herrera,

Felipe. Presentación clínica aguda inusual del síndrome de Guillain Barre: A propósito de un caso. Ciencia e Innovación en Salud. 2018; e60:1-6.

DOI 10.17081/innosa. 60

Guillain Barré syndrome (GBS), described in 1916 by Charles Guillain

(Kusunoki, 2015), is currently the most frequent cause of flaccid paralysis in the

world and constitutes a neurological emergency (Nayak, 2017). It is related to

autoimmune response characterized by progressive paralysis of the extremities, acute

areflexia and cytosolic albuminous dissociation (CAD) in cerebro-spinal fluid (CSF)

(

Doctor, Alexander, Radunovic, 2018), eventually preceded by respiratory tract

infections (58%), gastrointestinal (22%) (De Wals, Deceuninck, Toth, Boulianne,

Brunet, Boucher, Landry, De Serres, 2012) even certain vaccines (Sejvar,

Baughman, Wise, Morgan, 2011). The GBS incidence is between 0.8-1.9 cases/

100,000 inhabitants (Van den Berg, Bunschoten, van Doorn, Jacobs, 2013)

Mortality can reach up to 5% of cases, despite treatment with immunotherapy or

plasmapheresis (Yuki, H.-P., 2012) within the forms of expression of GBS are described

clinically variants associated with anti-ganglioside antibodies (GM1, GD1a, GT1a,

GQ1b) including: Acute inflammatory demyelinating polyneuropathy and its facial

variant (diplegia and facial paresthesia), acute motor axonal neuropathy and its extensive

form (acute motor sensory axonal neuropathy, multifocal neuropathies due to acute

conduction block), pharynx-cervical-brachial variant, Miller Fisher syndrome, its incomplete

form (acute ophthalmoparesis without ataxia), acute ataxic neuropathy (without

ophthalmoplegia) and an even more rare variant, Bickerstaff's brainstem (Etxeberria,

Lonneville, Rutgers, Gille, 2012).

II CASE REPORT

A 56-year-old male patient, Colombian, with a history of high blood pressure, who was

admitted to the emergency department due to clinical symptoms of 1 hour of evolution

characterized by paresthesia’s in the left side of the face with deviation of the labial

commissure to the right, weakness of the left cerebral brachium of progressive behavior; as

related, she reported an episode 15 days prior of liquid diarrhea without signs of dysentery

and uncalcified fever, symptoms that had spontaneous resolution without drug treatment.

Upon physical examination, there was an increase in blood pressure, dyspnea, afebrile,

alertness, oriented, with judgmental and conserved reasoning, fluent, coherent language,

adequate executive function, cranial nerves: with clinical evidence of paralysis of the left

seventh cranial nerve, Bell sign positive score House Brackmann: IV / VI. Not finding other

alterations of the cranial pairs. Dynamictaxi with evidence of a Parética march requiring

support, score Daniels for strength in the left upper limb 2/5, ipsilateral lower limb 3/5, right

hemibody 5/5, preserved surface and deep sensitivity, normal osteotendinous reflexes (++ /

++++), without pathological reflexes.

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I. BACKGROUND

Normal cerebral tomography (CT), laboratory tests, normal chest radiograph,

electrocardiogram without alterations. The diagnostic possibility of cerebrovascular event

was raised, however, after 5 hours of evolution, it presents dysphagia for fluids, areflexia,

generalized weakness and acute respiratory distress, it is transferred to the intensive care

unit (ICU) where it presents a ventilatory failure requiring orotracheal intubation.

On day 2 in the ICU, nuclear magnetic resonance (NMR) was performed, cervical, dorsal

and lumbar spine without abnormal findings. A lumbar puncture (LP) showed cerebro-

spinal fluid of normal characteristics. Given the clinical context, GBS was suspected,

neurophysiology studies were carried out, confirming the diagnosis. On the 3rd day of stay

in ICU plasmapheresis begins. On the 4th day the progression continues in the

neurological deterioration conditioning an enclosure syndrome maintaining communication

through the ocular movements managing to manifest conservation of sensitive levels,

without changes at the motor level; On the 5th day, signs of an acute systemic

inflammatory response begin, a broad-spectrum antibiotic is initiated due to aspiration /

nosocomial pneumonia. On the 6th day of stay in the ICU after his third plasmapheresis

session, he had a torpid evolution, multiorgan failure, cardiorespiratory arrest and death.

Necropsy was not authorized by relatives

III. DISCUSSION

GBS is an acute polyneuropathy of autoimmune response with heterogeneous clinical

manifestations, which is the most frequent flaccid paralysis in the world (Webb, Brain,

Wood, Rinaldi, Turner, 2015). Most of the studies that estimate GBS incidence rates were

conducted in Europe and the USA, showing a range of 0.8-1.9 (median1: 1) cases /

100,000 persons per year. The annual incidence of GBS increases with age (0.6 / 100,000

children year and 2.7 / 100,000 people over 80 years), slightly more frequent in men than

women (Yuki, Kokubun, Kuwabara, Sekiguchi , Ito , Odaka , Hirata ,Notturno , Uncini,

2012) The case presented is consistent with a small percentage of patients described in

the world literature, in the case of a 56-year-old male patient with acute atypical

symptoms, asymmetric weakness with osteotendinous reflexes present. Studies have

succeeded in demonstrating GBS variability with normal or hyper-excitable

osteotendinous reflexes during the clinical course of the disease in approximately 10%

of patients (Fokke, van den Berg, Drenthen, Walgaard, van Doorn, Jacobs, 2014)

Scientific evidence associates respiratory or gastrointestinal tract symptoms up to 4 weeks

before the start of GBS (Musso, Cao-Lormeau, Gubler, 2015) and infectious

processes of microorganisms isolated in the laboratory such as Campylobacter jejuni,

cytomegalovirus, Epstein Bar virus, Influenza A, Mycoplasma pneumonia, HIV,

Zika and Chikungunya, among others (Wong, Umapathi, Nishimoto, Wang, Chan

& Yuki, 2015; Chaverra, & Ayala, 2017) cases of post-vaccine SGB has been

described in rabies virus, Influenza A, H1N1.

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In the documented case, it is mentioned that 15 days prior to the start of GBS, there were

gastrointestinal manifestations that followed a self-limiting course. Regarding the

symptomatic progression, the evidence shows an average of 1-2 weeks after the

immune trigger and advances to a clinical maximum of 2-4 weeks (Fokkin, Selman,

Dortland, Durmus, Kuitwaard, Huizinga et al, 2014). This point is interesting in the

presentation of the case, where the symptomatic progression is established in hours up to

approximately 4 days managing to severely compromise the patient, conditioning it to the

confinement syndrome.

The clinical diagnosis of GBS classically involves acute ascending, symmetric progressive

areflexia paralysis with albuminous cytological dissociation; an atypical presentation

constitutes a diagnostic challenge for the medical specialist given the symptomatic

heterogeneity and diverse diagnostic possibilities. In the case described, his clinical

presentation was atypical and manifested by left brachiocrural weakness, which later

became generalized and initially associated with normal tendon reflexes, in addition to a

normal CSF. According to the literature, there are studies that show the approximate time of

albumin-cytological dissociation in CSF from the week following the start of GBS, not

constituting a confirmatory or excluding test of GBS. 15% of patients with the disease have

an increase in the CSF cell count (5-50 cells μL) (Hughes, Swan, Van Doorn, 2012)

Finally, the treatment of GBS shows efficacy of both immunoglobulin and plasmapheresis,

directly related to early onset. In the case presented once the diagnosis was confirmed,

plasmapheresis was started, however, the rapid progression favored conditions causing

death.

IV. CONCLUSION

It constitutes a diagnostic challenge for the neurologist the atypical presentations of GBS,

especially those presenting with rapid progressive evolution, are a diagnostic challenge for

the neurologist. It is important to highlight the importance of suspecting this pathology in the

emergency department for timely diagnosis. Based on this case, it can be seen that the

form of presentation can hinder diagnosis and treatment in the early stages of the disease

and thereby delay the patient's chances of effective recovery. However, there is a

small percentage of patients who still have diagnosis and timely treatment, his disease

continues its natural course progressing to death

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