Beyond the Usual: A Case Series on Acute Hypokalemic Paralysis with a Rare Neurological Twist

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

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.

Case presentation: We present three male patients with different manifestations of multifocal motor neuropathy. Patient 1: 56-year-old male with history of right upper limb weakness starting at age 31, progressing to weakness in both upper limbs over two years, followed by weakness in the lower limbs with a distal predominance after three years. Motor nerve conduction studies showed conduction block and uneven reduction in conduction velocity in all four limbs. Myography revealed positive sharp waves, fibrillations, and rarefaction of motor units’ recruitment. Tests for GM1 antibodies were negative. Patient 2: 58-year-old male experiencing since the age of 45 weakness in the right lower limb, progressing within a year to bilateral weakness with drop feet. Nerve conduction studies indicated reduced motor conduction velocity in all four limbs with conduction block and no changes in the sensory conduction study. Myography showed fasciculations and rarefaction of motor units’ recruitment. Patient 3: 58-year-old male with a seven-year history of distal weakness in the upper limbs, accompanied by cramps and pain. The first motor conduction study was unremarkable, and myography indicated chronic and acute denervation in four limbs compatible with motor neuron disease. After 01-year, the nerve conduction study was repeated in our service, showing motor conduction blocks, while sensory conduction remained normal. Myography during voluntary contraction indicated signs of chronic reinnervation in the proximal and distal muscles, with rarefaction of motor units in all four limbs. Complete exome sequencing revealed an alteration in the TTN gene variant chr2: 178,560,016 G>A, unrelated to the clinical findings. All three patients underwent treatment with intravenous human immunoglobulin and showed satisfactory symptom response. The treatment began at 25, 13, and 7 years after the onset of symptoms, respectively. Discussion: Multifocal motor neuropathy (MMN) is an uncommon, purely motor neuropathy characterized by asymmetric deficits with a preference for upper limb involvement. However, it is crucial to emphasize the importance of electrodiagnostic examination in the presence of atypical clinical presentations of the disease. Final comments: Multifocal motor neuropathy is a treatable condition. However, it is challenging to diagnose and can be confused with motor neuron disease (ALS and its variants). Hence, early diagnosis and treatment are crucial for improving the prognosis. It is important to note that symptom improvement may occur after many years of illness.

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.

Stroke in children is associated with a multitude of risk factors compared to risk factors of adult stroke such as hypertension, diabetes or atherosclerosis. A 15-year adolescent girl presented with acute onset weakness involving right upper and lower limb. She complained of neck pain and fever 2 days before the onset of hemiparesis for which her parents took her to traditional healer who performed neck manipulation after which she developed vomiting, tingling numbness and weakness of right upper and lower limb. There was no history of preceding headache, ear discharge or any other contributory history. Clinical examination revealed Glasgow Come Scale 12/15, power of grade 2/5 in right upper limb and 3/5 in right lower limb, exaggerated deep reflexes, extensor plantar reflex, right sided ptosis and right sided upper motor neuron facial palsy. CT scan brain showed right cerebellar and occipital infarct with posterior inferior cerebellar artery territory involvement. MRI Brain and MR Angiogram showed wedge shaped infarct involving right posterior inferior cerebellum, inferior vermis, ventral aspect of superior medulla, paracentral pons, right cerebral peduncle, tectum of both halves of midbrain with no internal hemorrhage and no vessel abnormality and right vertebral artery was not visualized. Her coagulation profile and cardiac work up were normal. She was treated with antiplatelet drugs, anticoagulants and physiotherapy following which the child gradually improved over a period of one month. In this case, with a positive history of neck manipulation authors can conclude that the etiology of young stroke wasinduced byneck manipulation. This case has been reported to increase awareness about the ill effects of neck manipulation and counsel parents against performing such procedures for children. Early recognition of pediatric stroke is critical for immediate diagnosis, imaging and treatment with better outcomes.

BackgroundMyositis is a recognised complication of numerous systemic viral infections including influenza. In adults the typical pattern is characterised by myalgia and marked proximal muscle weakness in upper and lower limbs and resolves slowly over weeks rather than days.Case presentationHere, we describe two male patients with myositis with an unusual distribution of weakness in the distal upper limbs, which both followed a flu-like illness and resolved spontaneously. Both patients had moderate elevations in creatine kinase, extensive negative serological investigations, normal nerve conduction studies and myopathic changes on electromyography.ConclusionsIn the para-infectious context, myositis is an important differential of acute distal upper limb weakness. This unusual pattern of acute muscle weakness should be recognised to avoid unnecessary in treatments. Similar cases in the recent literature in male patients between the ages of 25 to 55 are reviewed and suggest an emerging pattern of para-infectious myositis.

Cervical cord infarction mimicking migraine in a patient with vertebral artery dissection.

Acute-Onset Oculobulbar and Proximal Weakness in a Patient With Crohn's Disease: A Clinical Vignette.

Context: The Spinal Muscular Atrophy (SMA) is a genetic disease responsible for degeneration of the motor neurons of the spinal cord and the motor nucleus of the cranial nerves. Case report: Male patient, 32 years old, 6 years ago presented proximal weakness in lower limbs and distal weakness in upper limbs with progressive worsening and episodes of dyspnea on physical exercise, without dysphagia and dysarthria. The lower limbs showed grade of power of 3/5 in the proximal muscle group and 4/5 in the distal muscle group. On the upper limbs the grade of power was 4/5 in both proximal and distal group of muscles. Patellar tendon reflex and the bicipital reflexes were intact. Proximal lower limb atrophy, poliminiclonus in upper limbs, and the examination of sensitivity and cranial nerves were unremarkable. Electroneuromyography showed lower motor neuron involvement. MRI of cervical and lumbar spine was normal. Genetic test with homozygous deletion in the SMN1 gene, with four copies of the SMN2 gene. The patient was unable to perform spirometry due to the worsening of the respiratory condition. BIPAP requested, which improved the patient’s condition. Conclusions: There is poor information on literature about the lung function with the disease in adults, with no longitudinal study of the lung function in patients with Spinal Muscular Atrophy 3b and 4. Our case report demonstrates the importance of attempting respiratory symptoms even in adult patients with SMA.

Introduction: Guillain-Barre Syndrome (GBS) is an antibody-driven post-infective autoimmune disorder that typically presents with symmetrical ascending weakness. Other than the classical GBS, there are well-known GBS variants that have characteristic features. Here we present a case with an atypical presentation, where acute weakness and areflexia started from the right upper limb, descended to the lower limbs, and finally involved the left upper limb Case Presentation: A 42-year-old previously healthy male patient presented with acute onset right upper limb weakness that rapidly progressed in a descending manner to the lower limb and later to the left upper limb over several days. Later in the progression, respiration was also partially involved. However, other functions such as swallowing, eye movements cerebellar functions, and sensory functions were intact. MRI brain and other basic investigations were normal. MRI brain and cervical spine were normal, and nerve conduction studies showed acute motor axonal neuropathy type GBS. The patient completely recovered with IV Immunoglobulin treatment and physiotherapy without residual motor deficit.Conclusion: GBS is a heterogeneous disease with various presentations, and many rare presentations remain unstudied. Therefore, it is important to suspect GBS in any type of flaccid progressive paralysis unless proven otherwise, especially in resource-poor settings. Early detection and treatment prevent long-term motor deficits and other complications.

BackgroundThe aim of this study is to describe the different forms of polyneuropathy associated with coronavirus disease 2019 (COVID-19) as a secondary neurological complication for (COVID-19) and the outcome from different therapeutic regimens in adults and pediatrics in first and second waves of the pandemic.Case presentationThis study was conducted on 42 patients, they were divided into two groups, group (A) and group (B) in first and second waves respectively. Twenty-five patients presented by ascending weakness preceded by fever, dry cough and respiratory distress, electromyography (EMG) and nerve conduction (NC) studies done and confirmed the clinical diagnosis of demyelinating polyneuropathy. Eight patients presented by acute flaccid quadriparesis, more severe in upper limbs preceded by fever and diarrhea diagnosed as acute axonal polyneuropathy. Five patients presented by severe fatigue and progressive weakness of both lower and upper limbs, they developed fever and cough 10 days after the neurological symptoms. EMG and NC done and confirmed clinical diagnosis of polyneuropathy of demyelinating with secondary axonal picture. Four patients presented 30 to 40 days after their recovery form corona virus infection with gradual progressive weakness of both upper and lower limbs over 2 to 3 months duration, mainly the proximal muscles of lower limbs were affected with areflexia. EMG and NC done and confirmed the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).ConclusionWe should gain a better understanding of the underlying pathophysiology and therapeutic options of polyneuropathies related to COVID-19, which will have an impact on the treatment of the COVID related respiratory failure presenting with neuropathy.

IntroductionCeliac disease (CD) or gluten-sensitive enterophaty is an autoimmune disease of the small intestine precipitated by the ingestion of gluten in genetically susceptible individuals.The prevalence is 1 of every 120...

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.

IntroductionIn Charcot-Marie-Tooth type 1A (CMT1A) patients, daily life is mainly influenced by mobility and ambulation dysfunctions. The aim of our work was to evaluate the perception of disturbances that mostly impact on daily life in CMT1A patients and its difference on the basis of age, gender, disability, and quality of life.MethodsForty-one CMT1A patients underwent neurological assessment focused on establishing clinical disability through the Charcot-Marie-Tooth Neuropathy Score (CMTNS) and quality of life through the Short Form-36 (SF-36) questionnaire. We identified from CMT disturbances 5 categories [weakness in lower limbs (WLL), weakness in upper limbs (WUL), skeletal deformities (SD), sensory symptoms (SS), balance (B)] and patients classified the categories from the highest to the lowest impact on daily life (1: highest; 5: lowest). Ranking of the 5 categories, in the overall sample and in the different subgroups (dividing by gender, median of age and disease duration, CMTNS, domains of SF-36), was obtained and differences among subgroups were assessed using a bootstrap approach.ResultsRank analysis showed that WLL was the most important disturbance on daily life whereas WUL had the lowest impact. In the older CMT1A group, the most important disturbance on daily life was B that was also the most relevant disturbance in patients with a greater disability. SD influenced daily life in younger patients. SS had less impact on daily life, with the exception of patients with a milder disability.DiscussionOur findings demonstrated that the perception of disturbances that mostly impact on CMT1A patients’ daily life changes over the lifetime and with degree of disability.

Background Neuromyelitis optica is a severely disabling inflammatory disorder of the central nervous system of autoimmune etiology that mainly affects the optic nerves and spinal cord. Here, we present a case report detailing a patient with tingling and weakness of right upper and lower limbs who was neuromyelitis optica immunoglobulin G-positive. Case Presentation A 46-year-old Nepalese man presented to the hospital with a history of tingling and weakness of right upper and lower limbs that developed over a period of two months. Clinical evaluation showed diminished power across all major muscle groups in the right upper and lower limbs. Magnetic resonance imaging of his cervical spine showed T1 iso- to hypointense signal and T2 hyperintense signal in central cervical spinal cord from first to sixth cervical level, probably suggestive of myelitis or demyelination. The patient was immediately started on intravenous methylprednisolone. The diagnosis of neuromyelitis optica was later confirmed with strongly positive neuromyelitis optica immunoglobulin G. Conclusion In resource limited setting, in the absence of tests for neuromyelitis optica immunoglobulin G, treatment was started and the patient's condition started to get better. Hence, early initiation of aggressive immunosuppressive treatment is essential in such cases.