Miller Fisher syndrome and Guillain-Barré syndrome: dual intervention rehabilitation of a complex patient case.

Purpose: Guillain-Barré syndrome (GBS) presents with acute peripheral neuropathy leading to ascending motor and sensory deficits. Miller Fisher syndrome (MFS), a GBS variant, is characterized by ophthalmoplegia, ataxia, and areflexia. In unusual cases, MFS and GBS overlap. The purpose of this case report is to illustrate the effects of an aquatic and land-based physiotherapy (PT) intervention on a patient with MFS-GBS.Case Description: A 57-year-old male physician was diagnosed with complex regional pain syndrome following a quadriceps muscle tear. Within 1 month, the patient experienced evolving motor, sensory, autonomic, and cranial nerve dysfunction and was diagnosed with MFS-GBS.Interventions: Five months post-onset, a 7-week intensive PT program was initiated including aquatic and land-based interventions.Outcomes: Following completion, functional improvements were demonstrated on the 6 Minute Walk Test, Timed-Up-and-Go, 10 Meter Walk Test and Short Form-36. However, 6 weeks after program completion, the patient had a recurrence.Conclusion: PT intervention demonstrated improvement in functional outcomes for a patient with a diagnosis of MFS-GBS. Complex patients lacking recovery within 6 months may benefit from continued rehabilitation. Other intervention approaches may need to be considered, including aquatic therapy.

Wernicke's encephalopathy post hyperemesis gravidarum misdiagnosed as Guillain-Barre syndrome: lessons for the frontline.

We report a case of a 26-year-old pregnant woman, who presented with subacute limb weakness. This was initially suspected to be Guillain-Barre syndrome but subsequently found to be the motor neuropathy of dry beriberi (vitamin B1, thiamine deficiency) along with associated Wernicke's encephalopathy (WE). The underlying cause was revealed as hyperemesis gravidarum (HG). HG complicates up to 3% of pregnancies and if severe, without nutritional supplements, may lead to electrolyte disturbances, calorie loss and vitamin deficiency. Although the association of HG and WE was first reported in 1939, it remains an under diagnosed condition with potential for serious and permanent neurological deficits, and some mortality, in both mother and baby. Early recognition of the problem, with timely and careful fluid, electrolyte, glucose and vitamin replacement is needed to avoid complications. We highlight current best practice in the treatment of WE. An open mind to the possibility of HG complications in any pregnant woman presenting with neurological symptoms is probably the most important lesson to learn from the front line.

Severe Guillain-Barré syndrome associated with chronic hepatitis B: A case report and literature review.

RATIONALE: Guillain-Barré syndrome (GBS) is a postinfectious autoimmune peripheral neuropathy characterized by acute paralysis of the limbs. Clinically, extrahepatic manifestations of neurologic involvement in chronic hepatitis B (CHB) are uncommon. Little attention has been paid to the relationship between GBS and CHB viral infection. PATIENT CONCERNS: We presented a severe case of a 34-year-old man with general fatigue, anorexia, jaundice, numbness, and even muscle atrophy in the limbs, and respiratory failure during an acute exacerbation of CHB. DIAGNOSES: Serological liver enzymes test confirmed an acute exacerbation of CHB. Nerve conduction studies revealed the features of acute motor and sensory axonal neuropathy combined with acute inflammatory demyelinating polyneuropathy, and cerebrospinal fluid analysis showed albuminocytologic dissociation. Clinical manifestations and the test results were consistent with a diagnosis of severe CHB-related GBS. INTERVENTIONS: He was treated with mechanical ventilation, 2 courses of intravenous immunoglobulin, antichronic hepatitis B drugs therapy supplemented by hepatoprotection, acupuncture and rehabilitation. OUTCOMES: After 29 days of hospitalization, his neurological condition improved. At a 6-month follow-up visit, he was able to walk with the support of another person. LESSONS: The acute exacerbation of CHB may be a potential predisposing factor for the onset of GBS. This case is a reminder to clinicians that during the acute exacerbation of CHB, patients with neurological symptoms in the limbs should be considered for potential CHB-related GBS.

Dry Beriberi Due to Thiamine Deficiency Associated with Peripheral Neuropathy and Wernicke's Encephalopathy Mimicking Guillain-Barré syndrome: A Case Report and Review of the Literature.

BACKGROUND Beriberi due to thiamine (vitamin B1) deficiency has two clinical presentations. Patients with dry beriberi present with neuropathy, and patients with wet beriberi present with heart failure, with or without neuropathy. Dry beriberi can mimic the most common form of Guillain-Barre syndrome (GBS), an acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Severe thiamine deficiency results in Wernicke's encephalopathy. This report of a case of dry beriberi and Wernicke's encephalopathy due to thiamine deficiency includes a review of the literature. CASE REPORT A 56-year old woman with a history of gallstone pancreatitis and protein-calorie malnutrition was treated six months previously with total parenteral nutrition (TPN). She initially presented at another hospital with paresthesia of the lower limbs, arms, and neck, and symptoms of encephalopathy. Initial diagnosis of GBS was made, based on magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) findings. Despite five days of intravenous immunoglobulin (IVIG) treatment, her encephalopathy worsened, requiring transfer to our hospital, where she required intubation and treatment with vasopressors. A repeat MRI of her brain showed changes consistent with Wernicke's encephalopathy. Following treatment with high-dose intravenous thiamine, her mental status improved within 48 hours, and by the third hospital day, she no longer required intubation. CONCLUSIONS Symptoms and signs of dry beriberi due to thiamine deficiency can mimic those of acute or chronic GBS. However, thiamine repletion leads to rapid clinical improvement and can prevent irreversible neurologic sequelae, including Korsakoff syndrome. Clinicians should consider thiamine deficiency in malnourished patients presenting with symptoms and signs of GBS.

Acute flaccid paralysis: Do not forget beriberi neuropathy.

We aimed to elucidate characteristics of beriberi neuropathy (BB) in a general hospital (GH) setting. Nerve conduction studies (NCS), cross-referenced with clinical records of patients admitted to a GH (May 2011-July 2017), were reviewed for diagnosis of BB. Thirteen patients (age range 23-64 years; five women) were diagnosed with BB. Eleven were incarcerated (2-24 months) at time of index event. Eleven reported prior, severe anorexia (2-6 months); five reported significant weight loss, three had recurrent vomiting, and three reported alcohol misuse. Commonest presentation was weakness (12/13); nine had symptom evolution over ≥3 weeks. At nadir, 11/13 could not walk independently. Other features included numbness/paraesthesiae (10/13), dysautonomia (6/13), vocal cord dysfunction/dysphagia (4/13), nystagmus (3/13). Pain was not prominent. Cerebrospinal fluid, tested in five patients, was acellular; one showed mildly increased protein. NCS showed predominantly sensorimotor, axonal polyneuropathy, rarely asymmetric. Only one patient had sural-sparing pattern. All received high dose thiamine. Two of the thirteen received intravenous immunoglobulin for suspicion of Guillain-Barré syndrome (GBS). Eleven improved to independent ambulation. One patient died from pulmonary embolism; one was lost to follow-up. Two of the thirteen had residual neurocognitive effects; both misused alcohol. Besides GBS, BB is an important cause of acute to subacute flaccid paralysis, especially in incarcerated patients and those with significant dietary deprivation. Features favoring BB over GBS are ≥3 weeks of symptoms, nystagmus, confusion, vocal cord dysfunction, volume overload, normal spinal fluid, elevated lactate, and absence of sural-sparing pattern in NCS.

Acute-onset multiple acyl-CoA dehydrogenase deficiency mimicking Guillain-Barré syndrome: two cases report.

BACKGROUND: Multiple acyl-CoA dehydrogenase deficiency (MADD) showed great clinical heterogeneity and poses a challenge to diagnosis. Guillain-Barré syndrome (GBS) is an acute-onset autoimmune-mediated peripheral neuropathy. However, no patients of acute-onset MADD mimicking the GBS phenotype are reported previously. CASE PRESENTATION: Two patients displayed acute-onset limb weakness, areflexia, and length-dependent sensory disturbances, which clinically indicate the diagnosis of GBS, but electrophysiological and cerebrospinal fluid results threw doubtful points to the initial diagnosis. The muscle biopsy showed lipid storage disorder; and compound heterozygous mutations in the electron transfer flavoprotein dehydrogenase (ETFDH) gene were found in the two patients through targeted next generation sequencing, which provided the definite diagnostic evidences of late-onset MADD. Muscle weakness was quickly improved by riboflavin supplementation, but sensory disturbances required a long-term treatment. DISCUSSION: The present two cases have demonstrated that MADD can mimic GBS. Taking into consideration the significant differences of therapeutic regimen and prognosis, MADD should be included in the differential diagnosis of GBS.

A Case of Mercury Toxicity Complicated by Acute Inflammatory Demyelinating Polyneuropathy.

A 13-year-old African American male presented with 2 months of subacute altered mental status, ptosis, areflexia, disordered gait, constipation, weight loss, abdominal and testicular pain, and hyperhidrosis. Initial workup at our facility was unrevealing until elevated serum mercury level was detected. Diagnosis of mercury toxicity was confirmed, and chelation therapy with succimer was started. After beginning succimer, the patient developed acute-onset weakness and was diagnosed with acute inflammatory demyelinating polyneuropathy. Supportive studies included elevated cerebrospinal fluid protein and acquired demyelinating polyneuropathy on nerve conduction study. He responded well to treatment with intravenous immunoglobulin and returned to his baseline state of health. Although there is a known association between mercury toxicity and axonal neuropathy, there is only 1 other case report of acute inflammatory demyelinating polyneuropathy in the setting of mercury toxicity. The nature of the correlation between these 2 entities in our case remains unclear.

Guillain-Barré syndrome during adalimumab therapy for Crohn´s disease: coincidence or consequence?

We report the case of a 64-year-old patient diagnosed with extensive ileal Crohn´s disease who developed Guillain-Barré syndrome after starting biological therapy with adalimumab. Neurologic involvement associated with inflammatory bowel diseases is recognized as an extra-intestinal manifestation. After the breakthrough of antitumor necrosis factor alpha (anti-TNF-α) agents, an increasing number of cases of acute inflammatory demyelinating polyneuropathies have been reported; however, only one case has been described in a patient with Crohn´s disease. Although a causal relationship between Guillain-Barré syndrome and TNF-α antagonist therapy cannot be proven, this report emphasizes the need to monitor for neurologic signs and symptoms in patients with inflammatory bowel diseases, with or without biological therapy, to avoid severe and irreversible complications associated with demyelinating diseases.

[Neurophysiological methods in evaliuation of neurorehabiltation in children]. / Przeslanki neuroelektrofizjologiczne dla rehabilitacji neurologicznej dzieci.

The authors reviewed neurophysiological methods, which are used in the evaluation of children referred for neurorehabilitation. Rehabilitation techniques which may stimulate or provoke pathological changes in EEG must be ruled out. Electrophysiological and clinical improvement allow for the extension and intensification of rehabilitation. Normal EEG pattern ensures the safe use of techniques consisting of neuromuscular re-education or passive verticalisation, electrotherapy and thermotherapy. Quantitative and qualitative assessment of cognitive impairment is based on neuropsychological tests and endogenous evoked potentials (most often P300). Presence of cognitive dysfunction needs the use of neuropsychological and neurologopedic therapy. Based on results of exogenous evoked potentials appropriate neurorehabilitation program (physiotherapy, kinezytherapy) can be determined and clinical outcome predicted. EMG allows appropriate usage of applications, patterns and principles in the PNF method (such as compression, stretching, resistance), adapting them optimally to the possibility of a child. ENG estimates conduction in motor and sensory nerves. Based on the results nerve impairment can be localized, severity and character of damage estimated (demyelinating, axonal or complex) and course of the disease and treatment monitored. Short characteristics of 37 children with Guillain-Barre syndrome referred for rehabilitation was presented. Special attention was drawn to floppy infants. Results of neuroelectrophysiological examinations determine suitable rehabilitation program adjusted to the course of central nervous system impairment.

Guillain-Barré syndrome mistaken for a common spinal disorder: a case report.

CONTEXT: Many patients with musculoskeletal issues seek traditional Korean medicine or complementary and alternative medicine (CAM) to treat or help deal with their health conditions. Practitioners and patients should be aware of diseases that can exhibit similar symptoms, consequently complicating diagnosis and treatment. OBJECTIVE: The study intended to examine a case of Guillain-Barré syndrome (GBS), in which the patient presented with back pain, leg pain, and weakness that gave cause for diagnostic error at onset. Design • The research team has reported a case study. SETTING: The study was conducted at Jaseng Hospital of Korean Medicine in Seoul, Korea. PARTICIPANT: After initially being prescribed back surgery for lumbar disc herniation, as confirmed by lumbar magnetic resonance imaging (MRI) at a previous hospital, a 54-y-old female with low back and leg pain as her chief complaint chose to transfer to a Korean medicine hospital for nonsurgical treatment. INTERVENTION: The patient became nonambulatory owing to a sudden decrease in muscle strength during admission and underwent a cervical MRI to assess for cervical myelopathy because she developed additional neck pain and upper-limb symptoms. She then was diagnosed with GBS and received a regime of symptomatic treatment with CAM, including acupuncture, electroacupuncture, pharmacopuncture, and herbal medicine. RESULTS: The cervical MRI results showed that the symptoms were not due to myelopathy or radiculopathy, and GBS was suspected because of the progressive ascending weakness, absence of myotatic reflexes in the lower limbs, and preceding flu-like symptoms. The patient showed swift recuperation after the CAM treatments, recovering motor strength and ambulation. CONCLUSIONS: A seemingly typical patient with musculoskeletal symptoms may turn out to have a completely different pathology, and clinicians should keep such potential confounders and comorbidities in mind when diagnosing patients.

Pediatric lumbar puncture and cerebrospinal fluid analysis.

BACKGROUND: Lumbar puncture (LP) is a commonly performed procedure in pediatrics. Accurate analysis of cerebrospinal fluid (CSF) profile is essential in diagnosing and managing a variety of infectious and inflammatory conditions involving the brain, meninges, and spinal cord. It can also provide useful diagnostic information in the evaluation of possible subarachnoid hemorrhage and demyelinating syndromes, and aid in the diagnosis and management of pseudotumor cerebri. OBJECTIVES: To review anatomic, physiologic, and pathologic aspects of performing pediatric lumbar puncture and CSF analysis. DISCUSSION: Although still a commonly performed procedure in the outpatient setting, effective vaccines to prevent invasive infection due to Streptococcus pneumoniae and Haemophilus influenzae type b have greatly reduced pediatric bacterial meningitis rates due to these pathogens, resulting in decreased opportunity for physician-trainees to perfect this important skill (among nonneonates) during the 3 years of supervised residency training. Success in performing pediatric LP is augmented by a thorough understanding of medical aspects related to this procedure. This article discusses technical aspects involved in successfully performing a lumbar puncture to obtain CSF, and interpreting a CSF profile in children. CONCLUSION: A thorough understanding of anatomic, physiologic, and pathologic considerations regarding performing lumbar puncture and CSF analysis can augment success in diagnosing a variety of potentially serious pediatric conditions.

A Guillain-Barré syndrome-like neuropathy associated with arsenic exposure.

OBJECTIVES: We report on a patient presenting with an isolated polyneuropathy mimicking Guillain-Barré syndrome (GBS) associated with arsenic exposure. CASE: A 43-year-old man visited our emergency room complaining of progressive quadriparesis over the prior 5 days. His clinical course with laboratory data was typical of GBS. However, because of his recent use of herbal medication, we screened for the presence of several heavy metals. Serial analyses of urinary inorganic arsenic concentrations confirmed exposure to arsenic. He was diagnosed as arsenic neuropathy mimicking GBS without any systemic manifestation of arsenic intoxication. CONCLUSIONS: The present case study emphasizes the need to consider arsenic intoxication in patients presenting with acute demyelinating neuropathies and histories of herbal medication use.

[Subacute inflammatory demyelinating polyradiculopathy associated with Epstein-Barr viral infection: a clinical case].

The clinical case of treatment a patient for subacute inflammatory demyelinating polyradiculopathy associated with Epstein-Barr viral infection is presented. It is showed that the period of rehabilitation after the indicated disease lasts long enough. It requires the differentiated approach in acute and recovery treatment period with using of different rehabilitation measures.

Guillain-Barre syndrome: management and treatment options for patients with moderate to severe progression.

Guillain-Barre syndrome (GBS) is a syndrome that affects the immune system and attacks the peripheral nervous system. Discussion includes defining GBS as well as its etiology and differential diagnosis. Patients with GBS are not uncommon, and therefore it is important to be educated and to have a more precise understanding. GBS patients need to be treated holistically through emotional and physical support and known effective treatments. Through this article, readers will be able to achieve a thorough understanding of GBS and management options/strategies. Clinical features and manifestation of presenting symptoms will assist in determining initial laboratory studies, imaging, and any other testing that should be performed. Proper and quick diagnosis of GBS will be critical to further optimize treatment options and to decrease the likelihood of further immediate progression. Treatment modalities will be discussed as well as management during the acute hospital course and after discharge from the acute care facility. Discussion will focus on moderate to severe cases and associating treatment plans evaluated from evidence-based practice.

Dry beriberi mimicking the Guillain-Barre syndrome.

A 44-year-old man is described with severe flaccid quadriparesis that evolved over 3 weeks. He had regularly binged on alcohol-up to 20 cans of beer per day with occasional consumption of spirits-for more than 15 years but had balanced this with regular food intake. However, for a week prior to the current episode he had not eaten anything of significance. Nerve conduction studies revealed a background peripheral, mainly sensory, neuropathy with a superimposed acute motor axonopathy. CSF was normal. He improved with high dose vitamin replacement and physiotherapy but remains dependent on a Zimmer frame for mobility and a splint for wrist drop.

Síndrome de Miller Fisher como presentación de una encefalopatía de Wernicke / Miller fisher syndrome as the presenting symptom of wernicke’s encephalopathy

La encefalopatía de Wernicke es un síndrome neuropsiquiátrico agudo secundario a un déficit de tiamina, definido por la tríada característica de confusión, oftalmoparesia y ataxia, aunque se han descrito presentaciones raras que retrasan el diagnóstico. El síndrome de Miller Fisher se caracteriza por la tríada de oftalmoparesia, ataxia y arreflexia,y se considera una variante del síndrome de Guillain-Barré; su diagnóstico diferencial incluye la encefalopatía de Wernicke. Caso clínico. Mujer de 75 años con trastorno digestivo crónico, que desarrolla un cuadro agudo de oftalmoplejía internuclearbilateral, ataxia y arreflexia, con disociación proteinocitológica en el líquido cefalorraquídeo, por lo que se propuso el diagnóstico inicial de síndrome de Miller Fisher. Los estudios neurofisiológicos fueron normales, los anticuerpos anti-GQ1b,negativos, y la resonancia magnética cerebral sugirió una encefalopatía de Wernicke; la respuesta a la tiamina fue espectacular. Conclusiones. Las similitudes en la distribución lesional de ambas entidades, en la semiología y los resultados analíticos, así como la influencia de ciertos factores de confusión (hiponatremia, edad avanzada), conformaron un síndrome típico que favoreció un diagnóstico de sospecha etiológico erróneo, que pudo modificarse precozmente a la luz de ciertas pruebas diagnósticasy después de la respuesta terapéutica. Además de tratarse de una presentación atípica para una encefalopatía de Wernicke, este caso recuerda que la conjunción del diagnóstico sindrómico con el etiológico pasa por realizar un correcto diagnóstico diferencial apoyándonos en detalles de la anamnesis, en las pruebas complementarias necesarias y en el seguimientoevolutivo de los pacientes, incluso cuando nos encontramos ante síndromes típicos habitualmente relacionados con una etiopatogenia predominante

Wernicke’s encephalopathy is an acute neuropsychiatric syndrome resulting from a thiamine deficit,which is defined by the characteristic triad of confusion, ophthalmoparesis and ataxia, although rare presentations have been reported that delay its diagnosis. Miller Fisher syndrome is characterised by the triad ophthalmoparesis, ataxia and areflexiaand is considered to be a variant of Guillain-Barré syndrome; its differential diagnosis includes Wernicke’s encephalopathy. Case report. A 75-year-old female with chronic digestive disorders, who developed an acute picture of bilateral internuclear ophthalmoplegia, ataxia and areflexia, with proteinocytologic dissociation in cerebrospinal fluid; accordingly, an initial diagnosis of Miller Fisher syndrome was proposed. Results of the neurophysiological studies were normal; anti-GQ1b antibodies were negative; and magnetic resonance imaging of the brain suggested Wernicke’s encephalopathy. The response to thiamine was spectacular. Conclusions. The similarities in the distribution of the lesions of the two conditions, in the signs and symptoms and the lab findings, as well as the influence of certain misleading factors (hyponatremia, advanced age), wentto make up a typical syndrome that favoured a wrong presumptive aetiological diagnosis. This was corrected at an early stage, however, in light of the results of certain diagnostic tests and after observing the therapeutic response. In addition to being anatypical presentation for Wernicke’s encephalopathy, this case highlights the fact that for there to be an agreement between the syndromic and aetiological diagnoses it is necessary to carry out a correct differential diagnosis based on details from thepatient’s history, on appropriate complementary tests and on the follow-up study of how the patients progress, even when we come across typical syndromes that are usually related to a predominant aetiopathogenesis

Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery.

Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involvement. After treatment with immunoglobulin and vitamin supplementation, rapid clinical and neurophysiologic recovery was observed. We describe the clinical and electroneuromyographic features of this case, stressing the difficulty of initial diagnosis, particularly in the differential diagnosis with Guillain-Barré syndrome. We discuss the importance of nutritional follow-up and the eventual indication of routine vitamin supplementation in these patients.

Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery / Polineuropatia axonal aguda com acometimento proximal predominante: manifestação neurológica incomum de cirurgia bariátrica

Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involvement. After treatment with immunoglobulin and vitamin supplementation, rapid clinical and neurophysiologic recovery was observed. We describe the clinical and electroneuromyographic features of this case, stressing the difficulty of initial diagnosis, particularly in the differential diagnosis with Guillain-Barré syndrome. We discuss the importance of nutritional follow-up and the eventual indication of routine vitamin supplementation in these patients.

A cirurgia bariátrica é freqüentemente indicada no tratamento da obesidade mórbida. Complicações previamente não relatadas têm sido associadas a essa cirurgia; dentre estas, as complicações neurológicas têm recebido destaque. Relatamos o caso de um homem de 25 anos de idade submetido a cirurgia gástrica para tratamento de obesidade mórbida que desenvolveu, dois meses após a cirurgia, fraqueza de predomínio proximal nos membros inferiores, de instalação aguda. A eletroneuromiografia demonstrou polineuropatia periférica axonal nos membros inferiores, de predomínio proximal. Após tratamento com imunoglobulina e suplementação vitamínica, apresentou rápida melhora clínica e neurofisiológica. Descrevemos as características clínicas e eletroneuromiográficas desse caso, destacando a dificuldade diagnóstica inicial, particularmente com relação ao diagnóstico diferencial com síndrome de Guillain-Barré. Discutimos a importância de acompanhamento nutricional e a eventual indicação de suplementação vitamínica de rotina nesses pacientes.