Fulminant Guillain-Barré syndrome with quadriplegia and total paresis of motor cranial nerves as a result of segmental demyelination

1995 ◽  
Vol 134 (1-2) ◽  
pp. 203-206 ◽  
Author(s):  
Adrian K.Y. Tan ◽  
Michael W.L. Chee
2020 ◽  
Vol 3 (1) ◽  
pp. 4-11
Author(s):  
Sarmad Al Hamdani ◽  
Fatema Yusuf Aljanabi ◽  
Maryam Isa Abdulrasool ◽  
Alaa Haitham Salman

Intravenous immunoglobulin (IVIG) has long been regarded as the first-line treatment for Guillain-Barré syndrome (GBS), with plasmapheresis only being reserved for severe cases or used as an additional therapy of unproven efficacy. Here, we present the case of a 9-year-old girl with acute motor axonal neuropathy (AMAN), a rapidly progressive subtype of GBS that caused her to fall into respiratory failure. The patient failed to show a response 10 days after starting IVIG, but showed rather quick improvement with plasmapheresis. She received a total of 5 sessions of plasmapheresis on alternate days over a course of 8 days. Before starting plasmapheresis, her muscle strength was 2/5 in both upper limbs and 1/5 in both lower limbs, and she was dependent on mechanical ventilation. Following the first session, her power improved from 2/5 to 4/5 in the upper limbs, and the gag and sucking reflexes were recovered. On day 3, after the second session was initiated, she was extubated successfully (having been on a ventilator for 2 weeks) and remained on continuous positive airway pressure for the next 48 h, after which she was on room air. In addition, she was having hypertension from the first day of the diagnosis (which was due to autonomic instability), which improved after clonidine to maintain her blood pressure. She was also initially having urinary retention, then was off Foley’s catheter. The patient was discharged from the hospital 2 weeks following the first session of plasmapheresis, with power grade 4/5 in both her upper and lower limbs. Her cranial nerves had recovered fully, and she was able to walk with aids.


2022 ◽  
Vol 7 (4) ◽  
pp. 326-333
Author(s):  
Madhavi Karri ◽  
Deepak Jacob ◽  
Balakrishnan Ramasamy ◽  
Santhosh Perumal

A novel coronavirus (COVID-19) pandemic is caused by severe acute respiratory syndrome coronavirus 2 (SARs-CoV-2). This pandemic has been globally alarming in the current period. Several neurological manifestations are reported occurring with the infection. Guillain barre syndrome (GBS) or acute onset inflammatory polyradiculoneuropathy has been among the frequent manifestations observed among them. To know the pattern and outcome of GBS in COVID-19 affected individuals. We have taken six individuals admitted with flaccid quadriparesis in the last two months. All were affected recently by COVID 19 infection, which RT PCR of the nasopharyngeal swab confirmed. The study participants have undergone nerve conduction studies and have been diagnosed with Guillain Barre syndrome using Brighton criteria. We did cerebrospinal fluid (CSF) analysis after admission. We initiated all patients on Intravenous immunoglobulin according to body weight (2g/kg divided over five days). We used the Barthel index score to assess the outcome of the individuals. We observed a mean duration of 18.25 days between the COVID-19 infection and the onset of symptoms. Apart from motor quadriparesis and sensory symptoms being in common, we also noticed cranial nerves and autonomic involvement. We made the diagnosis using the nerve conduction studies and Brighton criteria. After initiating intravenous immunoglobulin, all patients had a good outcome, and quality of life was better after two months of follow up. Guillain Barre syndrome is one of the neurological manifestations of COVID-19 and has a dramatic response with intravenous immunoglobulin and better outcome with treatment.


2020 ◽  
Vol 11 ◽  
Author(s):  
Qian Cao ◽  
Hong Chu ◽  
Xiujuan Fu ◽  
Jiajia Yao ◽  
Zheman Xiao ◽  
...  

Objective: Acute bulbar palsy plus (ABPp) syndrome is a rare regional variant of Guillain-Barré syndrome (GBS) characterized by acute bulbar palsy combined with other cranial symptoms or ataxia without limb and neck weakness. We aim to investigate characteristics of ABPp syndrome and analyze its nosological position within the GBS spectrum.Methods: A patient with ABPp syndrome was reported, and previous case reports of patients who met the criteria for ABPp syndrome from the literature were reviewed.Results: A total of 28 patients were included in our study. Median age was 32 years. Most of the patients (78.6%) were from Asia, and 75.0% had preceding infection. The main accompanying symptoms were ophthalmoplegia (85.7%), facial palsy (60.7%), and ataxia (50.0%). There existed asymmetric weakness in the form of unilateral facial palsy (32.1%) and ptosis (3.6%). Approximately half of the patients had albuminocytological dissociation. All the tested patients were seropositive for antiganglioside antibodies, of which the two most common were immunoglobulin G (IgG) anti-GT1a (77.3%) and anti-GQ1b (59.1%) antibodies. Over one-third of the patients who underwent electrophysiological assessment showed subclinical neuropathy beyond cranial nerves. The outcome was generally favorable as 89.3% of patients made full recovery within 5 months.Conclusion: The hitherto largest case series of ABPp syndrome advances our understanding of this disease. Serologically, the presence of IgG anti-GT1a and anti-GQ1b antibodies predicts and contributes to the disease. Phenotypically, ABPp syndrome is more prone to be a separate subtype of GBS than overlap of distinct subtypes and has the potential to complement current diagnostic framework of GBS.


Author(s):  
E. I. Tsoma

Neurological manifestations of COVID‑19 infection are caused by its effects on CNS (headache, dizziness, disturbance of consciousness, convulsions, etc.) and PNS (anosmia, ageusia, visual impairment, radiculo‑ and neuropathy). Guillain – Barré syndrome (GBS) is a rare autoimmune disease associated with damage to the peripheral nervous system. 40 — 70 % of cases are associated with a previous infection: cytomegalovirus, Epstein–Barr virus, Haemophilus influenzae type b, Mycoplasma pneumoniae, Campylobacter jejuni etc. The clinical characteristics of this condition are progressive muscle weakness, reduction or loss of tendon reflexes (hyporeflexia and areflexia), paresthesias, paresis of the cranial nerves. The diagnosis is based on clinical data, cerebrospinal fluid analysis (protein level, cytosis, antigangliosid antibodies), as well as electroneuromyography. Most patients with Guillain – Barré syndrome totally recover. However, the most dangerous and severe complication of acute inflammatory demyelinating polyneuropathy is paralysis of the respiratory muscles. About a quarter of patients require urgent treatment at intensive care unit with mechanical ventilation and/or tracheostomy. Mortality in Guillain – Barré syndrome can reach to 10 %. There have been several reports of COVID‑19‑related GBS in the world scientific medical literature during the last year, but more information about this association and its implications is still missing. The aim of this report was to analyze the available information about cases of Guillain – Barré syndrome associated with COVID‑19 infection, to compare different variants of this condition and to share our own experience in clinical management of such patient.


2021 ◽  
Vol 15 (10) ◽  
pp. 1507-1514
Author(s):  
Ana Flavia De Morais Oliveira ◽  
Luciana Guerra Gallo ◽  
Mábia Milhomem Bastos ◽  
Amanda Amaral Abrahão ◽  
Paulo Rufalco-Moutinho ◽  
...  

Introduction: Guillain-Barre Syndrome (GBS) is an acute immune-mediated polyneuropathy that compromises the peripheral and cranial nerves. It is characterized by rapid-onset paresthesia accompanied by progressive weakness in the lower extremities followed by symmetric ascending paralysis. Methodology: assessment of sensitivity to detect GBS between March 2017 and May 2019 in a public referral hospital, using the capture-recapture method based on the Chapman estimator and comparing three GBS data sources: the hospital-based sentinel surveillance system (VSBH), Human Immunoglobulin Dispensing Records System (RDIH), and Hospital Information System (SIH). Results: A total of 259 possible cases were identified (captured). Of these, 58 were confirmed and most resided in the Federal District. The VSBH showed the greatest sensitivity in case identification. The temporal distribution of cases showed periods with no cases identified, and more were registered during the rainy season from October to May, when high temperatures also occur. Conclusions: Increased circulation of arboviruses and gastrointestinal infections during the rainy season may explain the greater concentration of GBS cases. It is important to note that one-third of the cases identified in the different data sources do not converge, demonstrating that no single surveillance system is 100% effective. The severity and possible increase in cases related to GBS demonstrates the need for an improved surveillance system capable of monitoring and following-up cases involving neurological syndromes, regardless of the event preceding infection.


2020 ◽  
Vol 20 (2) ◽  
pp. 227
Author(s):  
Danah Aljaafari ◽  
Noman Ishaque

Guillain-Barré syndrome (GBS) is a heterogeneous disorder with a diverse clinical presentation ranging from weakness of certain body regions to tetraparesis with autonomic dysfunction and respiratory failure. Paraparetic GBS is a variant of GBS which is characterised by weakness limited to the lower limbs only. It is crucial to identify such topographical presentations, as a delay in diagnosis can lead to delayed initiation of specific treatment, which can negatively impact the outcome. We report a 29-year-old female patient who presented to the King Fahd Hospital of the University, Al Khobar, Saudi Arabia, in 2017 with rapid onset asymmetrical weakness of lower extremities associated with bladder dysfunction during the immediate postpartum period. The weakness spared cranial nerves and arms and imaging studies of the spine was unremarkable. Cerebrospinal fluid investigations showed cyto-albuminologic dissociation and nerve conduction studies showed features of demyelination. The patient was diagnosed with a paraparetic variant of GBS and treated with intravenous immunoglobulin. She had almost recovered completely at the two–month follow-up.Keywords: Paraparesis; Guillain-Barré Syndrome; Demyelination; Postpartum Period; Case Report; Saudi Arabia.


2021 ◽  
Author(s):  
Gabriel Santaterra Barros ◽  
Ana Paula Ramires Chiminazzo ◽  
Maria Luiza Ricarte Ruggeri ◽  
Maria Luisa Pelaes Stipp ◽  
Helen Maia Tavares de Andrade

Context: areflexia and hyporeflexia are mandatory clinical criteria for the diagnosis of Guillain-Barré Syndrome (GBS). However there are case reports in literature of GBS that exhibit hyperreflexia. Case report: Male patient, 38 years old, after weakness and pain in lower limbs, after exercise, for 4 days, without alteration of balance and sphincter. A week earlier, he had diarrhea and fever. Previous bariatric surgery. On examination: For both upper limbs, the grade of power was 5/5 in the proximal muscle group and 4/5 in the distal muscle group. For the lower limbs, the grade of power was 4/5 in both proximal and distal muscle groups. The deep tendon reflexes were brisk (+2) throughout all four limbs. Patellar tendon reflex had bigger response (+3). Babinski’s and Hoffmann’s signs were negative. Tactile, painful, vibrating sensitivity and cranial nerves examination were all intact. The presence of reflex in the four limbs with patellar hyperreflexia, made the GBS hypothesis to be disregarded. Laboratory: normal CK, TGO, aldolase, TGP, LDH and vitamin B12. Electroneuromyography (ENMG) revealed the diagnosis of AMAN. After ENMG, cerebrospinal fluid known as leukocytes: 3.1 mm³, proteins: 95.2 mg/dl. Human immunoglobulin (0.4 g/ kg/day) was prescribed for 5 days and the patient recovered in 3 months. Conclusions: The case reported by us shows the importance to take into account the GBS hypothesis in the presence of hyperreflexia, especially in axonal variants, to prevent the treatment from being mistakenly postponed.


2021 ◽  
Author(s):  
Etedal Ahmed A. Ibrahim ◽  
Khabab Abbasher Hussien Mohamed Ahmed ◽  
Elmuntasir Taha Salah

Abstract Background: Neurological manifestation and complications are common due to the coronavirus infectious disease COVID-19. It affects higher functions, cranial nerves and the motor system. It can lead to headaches, convulsions, mental and psychological changes like delirium and insomnia. Guillain Barre syndrome rarely occurs as a consequence of or in co-incidence with COVID-19. The authors report a case of Guillain Barre syndrome as an example of a success story in managing a complicated case of COVID-19 in an elderly male with signs of a poor prognosis. Case presentation: A previously healthy 70-year-old man presented (on the 25th of June, 2020) with a fever and cough followed by quadriplegia and facial weakness one week later. He tested positive for COVID-19, and a nerve conduction study revealed demyelinating neuropathy consistent with Guillain Barre syndrome. He received treatment in the form of intravenous immunoglobulin with marked improvement despite poor prognostic features. Conclusion: Patients with COVID-19 can present with any symptoms, including diseases of the nervous system and peripheral nerves such as Guillain Barre syndrome, which respond very well to IVIG treatment despite poor prognostic factors such as old age, gender, rapid onset of complete paralysis, lymphopenia and a ground-glass appearance on CT chest scans, which all existed in this case.


Author(s):  
G. Sonachand Sharma ◽  
Anupam Gupta ◽  
Raghavendra K. ◽  
B.P. Navin ◽  
Meeka Khanna ◽  
...  

AbstractNearly half of patients with Guillain-Barré syndrome (GBS) have cranial nerve involvement. Ocular muscle weakness or ophthalmoplegia occurs in ~10% of these patients. Patients presenting with bilateral ptosis, with or without ophthalmoplegia, is a rare finding. Anti-GQ1b antibody has been found in Miller Fisher syndrome and GBS with ophthalmoplegia variants. We report a case of GBS in a young boy presenting with rare presentation of tetraplegia, bilateral ptosis, and facial palsy, no ataxia but ophthalmoplegia with seronegative anti-GQ1b antibody. Patient showed recovery in cranial nerves involvement as well as with motor and functional recovery after 3 weeks of inpatient rehabilitation (Barthel Index score improved to 60/100 at the time of discharge from 20/100 at the time of admission). He was independent for most of the activity of daily livings including ambulation at the time of discharge from rehabilitation unit (p < 0.001).


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