Atypical Presentation of Guillain-Barre Syndrome

Rituximab-responsive Guillain–Barré syndrome following allogeneic hematopoietic SCT

Background. Follicular carcinoma thyroid usually metastasises to bone. Common sites of bone metastasis include skull and spine. Spinal metastasis are more common in the cervical region followed by dorsolumbar spine. Cervical extradural lesions present with progressive quadriparesis, sensory loss, dysautonomia, and respiratory distress. Typical Elsberg phenomenon in a cervical extradural lesion is rare. Elsberg phenomenon involves the involvement of ipsilateral upper limb, ipsilateral lower limb followed by contralateral lower limb and contralateral upper limb. Case presentation. We are reporting a case of 47-year-old lady presented with progressive quadriparesis of 1-month duration. Her weakness started in left upper limb followed by left lower limb, right lower limb and right upper limb weakness. She also had sensory loss below the level of C7. She had undergone near-total thyroidectomy for solitary thyroid nodule 14 years back and was on thyroid supplementation since then. Histopathology at that time was reported as follicular adenoma with Hashimoto thyroiditis. Her right upper limb power was grade 4- Left upper limb grade 1 right lower limb Grade 3, left lower limb grade 2 with hypertonia of both upper and lower limbs. She was evaluated with MRI Spine which showed a dumb bell-shaped extramedullary lesion involving the C5-C6 vertebra with significant cord compression and encasement of the left vertebral artery. USG neck showed left supraclavicular lymph node enlargement and small residual thyroid tissue in the left side of the thyroid. USG guided FNAC from the thyroid tissue and neck nodes were inconclusive. The patient underwent C4 and C5 laminectomy and subtotal excision from the cervical lesion. Histopathology was reported as metastasis from follicular carcinoma thyroid. Postoperatively patient limb power improved to grade 3 left upper and lower limbs and was discharged and later referred for radioiodine ablation Conclusion. Cervical extradural metastasis from follicular carcinoma thyroid can present with Elsberg syndrome even without any neck swelling even after decades of post thyroidectomy status for a benign aetiology. Laminectomy and decompression may lead to clinical improvement.

Objectives Electrophysiology plays a crucial role in Guillain-Barré syndrome (GBS) diagnosis and subtype classification. The aim of our study was to assess the potential role of distal compound muscle action potential (dCMAP) for early differentiation between acute inflammatory demyelinating polyneuropathy (AIDP) and axonal GBS. Methods We retrospectively reviewed the medical records of 24 subjects with AIDP and 18 subjects with axonal GBS. We built up receiver operating characteristic curves for total dCMAP duration and negative phase of dCMAP duration, in order to derive cut-off values able to differentiate between AIDP and axonal GBS. Results The total duration of dCMAP was significantly prolonged in AIDP compared to axonal GBS. AUCs, odds ratio and positive predictive values were higher for total duration than for negative peak duration. Nerve conduction parameters in the lower limbs were more sensitive than those in the upper limbs in distinguishing AIDP from axonal GBS. Discussion Total duration of dCMAP dispersion may capture an adjunctive component of distal demyelination, not measured by the more traditional parameters and may thus represent a useful tool for early differentiation between AIDP and axonal GBS.

Case presentation: A 67-year-old woman presented with intense pain and distal weakness of the lower limbs, associated with edema and arthralgia with difficulty walking for 2 months. He developed disproportionate and areflexic flaccid tetraparesis, weight loss and urinary retention. On examination, there was distal muscular atrophy of the four segments, disproportionate flaccid tetraparesis with muscle strength scores as follows: distal upper limbs -3 and distal lower limbs -2, according to the Medical Research Council Scale (MRC). Deep reflexes abolished (patellar, achilleus, biccipital and triccipital). Normal cranial nerves. He reported immunization against COVID-19 (Astrazeneca) 4 days before the onset of symptoms, with post-vaccination Guillain-Barré syndrome (GBS) as the diagnostic hypothesis. The electroneurographic study revealed a primarily axonal sensorimotor polyneuropathy, affecting nerves in the upper and lower limbs, compatible with the axonal variant of GBS. Human immunoglobulin 0.4g/kg/day was administered for 5 days, there was a slight improvement in muscle strength with muscle strength scores evolving to: distal upper limbs -4 and distal lower limbs -3 according to the Medical Research Council Scale (MRC). After 6 months, it progresses again insidiously with disabling pain, progressive, asymmetric quadriparesis, worse in the left upper limb and left lower limb, in addition to xerostomia, xerophthalmia, livedo reticularis in the lower limbs, scleritis of the right eye and oral ulcers. For investigation, serology was requested, resulting in positive ANCA, positive anti-Ro, positive anti-MPO, in addition to a ground-glass interstitial lung lesion. An evaluation was carried out in conjunction with rheumatology and ophthalmology, and, based on clinical characteristics and laboratory and imaging findings, she was diagnosed with Granulomatosis with Polyangiitis (GP) and Secondary Sjögren’s syndrome (SS), with sensory axonal polyneuropathy as the first symptom. engine. The patient continues to be treated with monthly cyclophosphamide, maintaining severe functional disability. Discussion: Early diagnosis of SS and GP remains a challenge given the complexity of clinical manifestations, in addition to the need for comprehensive immunobiological tests. In the case in question, it is worth highlighting that the neurological manifestations preceded the typical symptoms of the pathologies in question. This report allows us to discuss the clinical situation and immunological patterns of this condition and emphasizes the importance of comprehensive immunological testing for patients with GBS variants. Final comments: The association between GP and SS brings a high level of complexity to the clinical presentation and represents a challenging diagnosis. The specific temporal relationships and clinical presentation of this overlap syndrome can be difficult, and it is important to suspect this correlation given the early appearance of a polyneuropathy.

Introduction Background: Guillain-Barr e syndrome (GBS) usually presents with lower motor neuron (LMN) symmetrical weakness, areflexia, and hypotonia. GBS has rarely been reported with up going plantars’ response. We report a case of GBS with up going plantars during the course of the disease. Citation: Ahmad SF, Al-Bader SY, Hashel JY (2014) Guillain-Barr E Syndrome with Bilateral Extensor Plantar Reflexes. J Neurol Neurol Disord 1(1): 105 . doi: 10.15744/2454-4981.1.105 Case presentation: A 52-year-old woman developed acute paralysis with areflexia following a diarrheal illness. There was no fever at the time of the onset. She developed numbness and asymmetrical weakness of both lower limbs which progressed to involve the upper limbs bilaterally. There were no cranial nerves, respiratory or sphinecteric involvements. Few days later, the plantars’ became upgoing bilaterally, with absent of other upper motor neuron signs. Cerebrospinal fluid (CSF) analysis showed albuminocytological dissociation. Early Nerve conduction study (NCS) showed absent H-reflex dispersed F-waves with prolonged maximum F-latencies and M-amplitude showed no clear block. MRI brain and spine were unremarkable. She was treated with intravenous immunoglobulin (IVIG). Repeated NCS two weeks after the onset, definitely confirmed conduction block. She was able to walk with support three weeks after the onset. Conclusion: GBS should be considered as a differential diagnosis in a patient with acute quadriparesis, even if there is asymmetrical muscle weakness and extensor plantars’’ response. Guillain-Barre syndrome (GBS) is an acute autoimmune polyradiculoneuropathy. Clinical features include progressive, symmetrical ascending muscle weakness usually of more than two limbs, and areflexia with or without sensory, autonomic or brainstem involvements. Weakness is prominent in the lower limbs muscles compared to the upper limbs; there is absence of fever at the onset of neural symptoms [1]. Although, the diagnosis of GBS is based on clinical criteria, the presence of suggestive findings in the nerve conduction studies (NCS) or albuminocytological dissociation in the cerebrospinal fluid (CSF) analysis help to confirm the diagnosis [2].The involvement of the central nervous system (CNS) in the GBS is rare [3]. We report a case of GBS with asymmetrical weakness and up going plantars’ during the course of the disease. A 52-year-old female, known case of diabetes, hypertension, and hypothyroidism, was admitted with acute weakness of both lower limbs. She had history of diarrhea 4 days prior to presentation, which lasted 2 days and improved with symptomatic treatment. Then she developed numbness and weakness of both lower limbs that involved the left side more than the right. The weakness started distally and ascended up proximally. The weakness started to involve the upper limbs 4 days later. There were no cranial nerves, respiratory muscles involvement, nor sphincteric symptoms. There was no fever at the time of onset and no history of recent vaccination. On examination, there was weakness of both lower limbs with hypotonia. The weakness of both lower limbs was asymmetrical with proximal muscle power of 2/5 (Medical Research Council grading) in the left side and 4/5 in the right side, while the power of the distal muscles was 1/5 in the left side and 3/5 in the right side. Examination of the upper limbs showed weakness of distal muscles of 4/5. Deep tendon reflexes were absent all over, and the plantars’ were flexors bilaterally. Decrease in fine touch and temperature sensations were seen in L4, L5, S1 distribution of the left side with loss of joint position and vibration sense bilaterally. There was no cranial nerves involvement. The investigations showed normal total and differential leukocyte counts, erythrocyte sedimentation rate (4 mm/h), creatine kinase level, and electrolytes (Na, Ca, K, Mg, phosphate). Vasculitis and paraneoplastic work up were all negative. CSF examination showed albuminocytologic dissociation. CSF sugar was 6.3 mmol/L (plasma glucose 11 mmol/L), protein was 1517 mg/L, and there are only 2 cells (100% lymphocytes) were detected. All causes of

Guillain–Barre syndrome (GBS) is an acute onset, usually monophasic immune-mediated disorder of the peripheral nervous system. The term GBS is often considered to be synonymous with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), but with the increasing recognition of variants over the past few decades, the number of diseases that fall under the rubric GBS have grown to include axonal variants and more restricted variants, such as Miller Fisher syndrome (MFS) [Table 1].[1] Table 1 Guillain–Barre syndrome—clinical variants Epidemiology The reported incidence rates for GBS are 1–2 per 100,000 population.[2–4] The lifetime likelihood of any individual acquiring GBS is 1:1000.[5] The subtypes of GBS have different incidence rates in different parts of the world. In Europe and North America AIDP is dominant contributing to 90% of the cases. In contrast in China and Japan AMAN being the most common subtype.[6,7] The picture is intermediate when we look at other population. In Indian series the incidence of AIDP and AMAN are virtually equal although AMAN is more common in younger patients.[8] There seems to be a slight preponderance of AIDP in studies by Gupta et al[9] and by Meena et al (unpublished data from NIMS, Hyderabad). Available Indian literature indicates a peak incidence between June–July and Sept–October.[10] In western countries, GBS is common in the 5th decade,[11] but in India it occurs more commonly at a younger age.[10,12] GBS is equally common in men and women and can occur at any age. There is a male preponderance among the hospitalized population.[10,12]

BackgroundDengue is an arboviral infection that classically presents with fever, joint pain, headaches, skin flush and morbilliform rashes. The incidence of neurological symptoms and complications in dengue varies from 1 to 25 % that include encephalopathy, Guillain–Barre syndrome (GBS), acute motor weakness, seizures, neuritis, hypokalaemic paralysis, pyramidal tract signs, and a few more. Dengue fever as an antecedent infection in GBS is uncommon.Case presentationA 34-years-old Sri Lankan Sinhalese male presented with fever, headache and myalgia of 3 days and developed leucopenia and thrombocytopenia without evidence of haemoconcentration. The diagnosis of dengue fever was confirmed as he had positive dengue NS1 antigen test on the third day of fever. He made full recovery and was discharged after 4 days of hospital stay. Six days later, he presented with history of acute flaccid weakness of both lower limbs and upper limbs which was of progressive ascending nature. The electromyography had evidence of demyelinating neuropathy and cerebrospinal fluid showed albuminocytological dissociation. Subsequently, IgM for dengue virus was positive.ConclusionDengue is endemic in Sri Lanka. Post dengue Guillain–Barre syndrome is a potential neurological complications of this infection.

Introduction: Moyamoya disease (MMD) is characterised by progressive stenosis of distal ICA and the resulting hazy network of basal collateral vessels. Increase arterial stiffness in MMD is not well described. Case report: We describe a young patient ML, a 42-year-old Chinese female, presented to hospital in June 2015 with headaches, on a background of known hypertension diagnosed at the age of 28 years. Her systemic examination was normal. Investigations were unremarkable. She was followed in General Medicine (GM) clinic from 2015, which ruled out secondary causes of hypertension, including renovascular disease, renal artery stenosis, phaeochromocytoma and hyperthyroidism. ML was treated for presumptive essential hypertension. Over time, her BP remained stable on candesartan 16 mg and bisoprolol 2.5 mg once daily. She continued to have intermittent headaches. Recurrent ischaemic strokes and diagnosis of Moyamoya disease ML presented with intermittent right-sided facial numbness in February 2017. A magnetic resonance imaging (MRI) brain done showed no acute infarct or hemorrhage, but moderately severe narrowing of the left terminal internal carotid artery (ICA), associated with poor flow signals in both the A1 segment of the left anterior cerebral artery (ACA) and the M1 segment of the left middle cerebral artery (MCA). She was started on clopidogrel 75 mg and atorvastatin 40 mg daily. The second stroke occurred in March 2019, when ML presented with acute right upper and lower limb weakness, and right facial, upper and lower limb numbness. Magnetic resonance angiography (MRA) demonstrated severe stenosis at the left terminal ICA, also involving the left M1 and A1 segments; collateral flow was observed. The third stroke happened in April 2019, presenting with acute right-sided facial and upper limb weakness, with BP of 201/86mmHg. MRI and MRA of the brain revealed new acute infarcts scattered in the territories of the left MCA and ACA. A CT angiogram re-demonstrated severe steno-occlusive disease involving the left ICA T-junction and left M1 and A1 segments, with extensive basal collaterals. In light of these findings, Moyamoya disease (MMD) was diagnosed. ML underwent a left external carotid-internal carotid bypass in June 2019. An arterial stiffness study by syphgmoCor in June 2020 using markedly increased arterial stiffness. Conclusion: We present first and very rare case of hypertension in a young patient with MMD associated with increased arterial stiffness. Systemic vascular affection in MMD may be the cause of increased arterial stiffness.

Journal Article Guillain–Barré syndrome and Campylobacter jejuni infection Get access R.D.M. Hadden, R.D.M. Hadden Department of Neuroimmunology, Guy's, King's and St Thomas' School of Medicine, London, UK Search for other works by this author on: Oxford Academic Google Scholar N.A. Gregson N.A. Gregson Department of Neuroimmunology, Guy's, King's and St Thomas' School of Medicine, London, UK Search for other works by this author on: Oxford Academic Google Scholar Journal of Applied Microbiology, Volume 90, Issue S6, 1 June 2001, Pages 145S–154S, https://doi.org/10.1046/j.1365-2672.2001.01363.x Published: 01 June 2001

Monomelic amyotrophy (MMA) a variant of motor neuron disease, has the characteristic features of wasting and weakness usually confined to a single upper or lower limb occurring predominantly in young males and a benign outcome. Symptoms of increased sweating, coldness and cyanosis have been observed in a few patients. The objective was to evaluate the involvement of the sympathetic nervous system in MMA by measuring sympathetic skin response. Electromyography, motor and sensory nerve conduction studies were done in all the four limbs of 9 patients with atrophy of one upper limb. Stimulation at Erb's point, and above and below elbow was done to look for evidence of conduction block. The sympathetic skin response (SSR) was recorded in all the limbs of these patients. Wasting and weakness of right upper limb in 7 patients and left upper limb in 2 patients was seen. The mean age was 28.3+/-10.1 years. Twenty-five age matched (24.8+/-4.8 years) healthy subjects served as controls. The mean SSR latency in the affected upper limbs of 9 patients was prolonged compared to the 25 control subjects (1.51+/-0.07 s vs 1.42+/-0.19 s, P=0.03). The mean value of SSR latency in 18 upper limbs of the 9 patients which included atrophied and unatrophied limbs was also prolonged compared to the controls (1.50+/-0.08 s vs 1.42+/-0.19 s, P=0.05). There was no significant difference of the mean latency of SSR between the atrophied upper limbs and the clinically normal upper limbs (1.51+/-0.07 s vs 1.49+/-0.09 s, P=0.51). The mean SSR latency in the lower limbs of the patients (2.09+/-0.09 s) did not significantly differ from the control subjects (1.97+/-0.28 s, P=0.09). Motor and sensory nerve conduction was normal and there was no evidence of conduction block. In MMA the sympathetic nervous system is involved in the atrophic upper limb and also in the clinically unaffected upper limb but not in the lower limbs.

BACKGROUND: Guillain–Barre syndrome (GBS) is a condition characterized by polyradiculoneuropathy associated with an immune-mediated response. Conventional therapies such as intravenous immunoglobulins and plasma exchange have been used to manage this condition, but they often result in unpleasant clinical and electrophysiological outcomes. The present case report describes traditional Ayurveda treatments, specifically Basti (medicated enema) and composite Ayurveda treatment, used to manage GBS. This report aims to provide insights into the effectiveness of traditional Ayurvedic treatments in managing GBS. MATERIALS AND METHODS: In this case, the patient presented with complaints of tingling and numbness in the bilateral upper and lower limbs for 2 months, weakness in bilateral upper and lower limbs for 1 month, inability to stand and walk, pain in the lumbar region, intermittent constipation, and dysphagia since 1 month. The patient was diagnosed with Guillain–Barre syndrome based on clinical findings, electromyography, and nerve conduction velocity test (EMG-NCV). He received treatment from a private hospital, but his symptoms were aggravated, so he was referred to Government Ayurveda Hospital Nagpur for further clinical management. Plasma transfusion was done at PVT Hospital 15 days before. He was treated based on the treatment principles of Vatavyadhi Chikitsa, Vata Dosha Upkram, and Santarpan Chikitsa. The primary goals of the therapy were to nourish the bodily tissues (Santarpan) and pacify the Pitta Dosha (Pittaghna). RESULTS: The patient’s electromyography and nerve conduction velocity test (EMG-NCV) results showed acute demyelinating sensorimotor polyneuropathy involving both the upper limbs and lower limbs. Compared to before treatment, EMG-NCV studies showed definite improvement in compound motor action potential (CMAP) amplitudes more prominently in upper limbs after 4 months of therapy. The muscle power grade increased from 0 to 5, indicating a significant improvement. The bedridden patient can walk alone after the completion of treatment. CONCLUSION: This case study reveals that Ayurvedic treatment has the potential for significant recovery in GBS instances.

Guillain-Barre syndrome (GBS) manifests clinically as an acute onset of generalized symmetrical and ascending weakness of the limb muscles with areflexia. Several variant forms of the disease with unusual distribution of muscles in- volvement have been described. Pharyngeal-brachial-cervical form of the disease is one of rare variants of GBS and only a few of cases have been reported in children. Here we report a 13-month-old boy, who developed acute weakness of pharyngeal, bulbar, cervical and upper limb weakness and the initial diagnostic work up were done to rule out acute brain stem stroke. We concluded that, while taking all other etiologies into consideration, pharyngeal-cervical-brachial variant of GBS should be considered in patient's symptoms with acute bulbar and upper extremity weakness in order to institute appropriate early management.

Autosomal dominant cerebellar ataxias are classified according to genetic subtype and collectively known as SCAs. Movement disorders including tremor, dystonia, parkinsonism, chorea, myoclonus, paroxysmal nonkinesigenic dyskinesia, stiff person-like syndrome, akathisia, myokymia, stuttering, tics, restless leg syndrome, spasmodic-like dysphonia, laryngeal stridor, and palatal tremor have been reported in some of the SCAs.1 They may be present at the disease onset or during the overall disease course and sometimes they may be present even before the development of ataxia.1 A 69-year-old male patient, presented with asymmetric onset tremulousness of his upper limbs for the last 15 years. There was progressive gait ataxia, head tremors, and involuntary, random, non-rhythmic, and flowing movements of his left upper limb for the last 3 years. There was clumsiness and frequent dropping of objects more with left hand due to these involuntary random and flinging movements and they were aggravated on walking and subsided during sleep. His past medical history was unremarkable. He had a family history of tremors of upper limbs and head tremor in his mother, eldest sister, and maternal aunt (Fig. 1i). His general and systemic examinations were normal. Extra-ocular examination showed square wave jerks. On motor examination tone and power were normal, deep tendon reflexes were brisk and bilateral plantars were extensor. His sensory examination was normal. He had head tremor and left upper limb chorea which was more severe when he extended his arm in an outstretched position. During walking the chorea was more severe. He also had mild ataxia and intention tremors in the upper limbs (See Video 1). His magnetic resonance imaging of the brain showed diffuse atrophy including the cerebellum (Fig. 1ii). Given his clinical presentation and strong positive family history, spinocerebellar ataxia (SCA) panel was ordered, and he was found to be positive for SCA12 (PPP2R2B gene) with 49 CAG repeats. Our patient had monochorea in the left upper limb which has been rarely reported in SCA12 patients. Huntington's Disease-like presentation in SCA 12 was earlier reported in an Italian family, where the index patient had choreo-athetoid movements along with ataxic and parkinsonian features within other family members.2 Choudhury et al., reported 21 cases of genetically diagnosed patients of SCA12 and found that tremor was the most common initial non-ataxic manifestation and none of their patients had chorea.3 Huntington's chorea-like presentation is more common in SCA17.4 Apart from SCA17, chorea is also reported in SCA2, SCA1, SCA3, SCA27, DRPLA, and SCA48.1, 4, 5 In SCA 27 chorea can be the sole manifestations, whereas in DRPLA a combination of chorea, myoclonus and dystonia is seen.1 SCA12 is mostly prevalent in specific ethnic groups “Agrawal communities” and our patient also belonged to this community.3 Although tremors are not specific to a given SCA, SCA12 is the only SCA in which action tremor is the presenting and most common sign. Also, very asymmetric postural and action tremor can occur in these patients.3 Our case illustrates that “Chorea” can be one of the clinical manifestations of SCA12 which is of utmost importance in a country like India since it is the second most common type of SCA.3 (1) Conception and design of the study; (2) Acquisition and analysis of data; (3) Drafting a significant portion of the manuscript and figures. SB: 1, 2, 3 CR: 1, 2, 3 SP: 1, 2, 3 The authors confirm that the approval of an institutional review board was not required for this work. We also confirm that the patient has given written informed consent for the publication of his video. We confirm that we have read the Journal's position on issues involved in the ethical publication and affirm that this work is consistent with those guidelines. No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work. The authors declare that there are no additional disclosures to report.

Dissociation between titer of anti-ganglioside antibody and severity of symptoms in a case of Guillain–Barré syndrome with treatment-related fluctuation

Methotrexate is an essential medicine used to treat childhood malignancies including acute lymphoblastic leukemia. Neurotoxicity manifesting as leukoencephalopathy is an important adverse effect of methotrexate. Methotrexate-induced leukoencephalopathy classically demonstrates sub-acute-onset neurological manifestations that include learning disability, progressive dementia, drowsiness, seizures, ataxia, and hemiparesis. These are rare in children and are generally reported following intrathecal or intravenous use of methotrexate. In contrast, acute onset neurotoxicity with oral use of methotrexate is very rare. We report a 10-year-old boy presenting with acute onset limb weakness and neurological signs due to methotrexate-induced leukoencephalopathy following oral methotrexate. A 10-year-old Sri Lankan boy presented with fever and headache for 5days and difficulty in walking for 2days. He was unable to stand unaided on admission, and his parents complained of repetitive, involuntary extension movements involving the right upper limb. He is a child diagnosed with acute lymphoblastic leukemia who was on treatment for a relapse with daily oral dexamethasone and mercaptopurine, weekly oral methotrexate and folinic acid, and once every two weeks intrathecal vincristine. On examination, he had dystonic movements of the right upper limb and hypotonia and reduced muscle power (grade 3/5) of the left upper and lower limbs proximally and distally. The muscle power of the right side was grade 4 (out of 5). Tendon reflexes were diminished in all four limbs, and the plantar response was flexor bilaterally. The child had dysmetria and intension tremors on both sides. T2-weighted magnetic resonance imaging of the brain revealed symmetrical high signal intensities with diffusion restriction involving bilateral putamen, subcortical areas, and deep white matter, suggesting treatment-related neurotoxicity due to methotrexate-induced leukoencephalopathy. Oral methotrexate was discontinued. He showed gradual improvement in limb weakness and other neurological signs following treatment with intravenous folinic acid, aminophylline, dexamethasone, and oral dextromethorphan. This case report describes a patient with rapidly progressing methotrexate-induced leukoencephalopathy following oral methotrexate. It highlights that the risk of neurotoxicity persists even with the oral use of methotrexate; therefore, the prescribers should be vigilant of this uncommon side effect.