Flaccid Quadriplegia Research Articles

Hyperreflexia in Guillain Barre Syndrome: A Case Report

Guillain Barre Syndrome (GBS) is an immune mediated polyradiculoneuropathy classically characterized by acute ascending type of motor weakness of limbs with areflexia but in AMAN variant of GBS preserved or exaggerated reflex sometimes can occur. We report a 45 year old female patient who presented with acute flaccid quadriplegia, bilateral facial lower motor type nerve palsy and bulbar involvement, initial hyperreflexia of all four limbs and bilateral plantar extensor response 7 days following an attack of diarrhoea. Sensory and bowel bladder function was intact. She was treated with IV immunoglobulin and IV methylprednisolone. Nerve Conduction study (NCS) revealed AMAN variant of GBS. All reflexes disappeared on the 2nd day onward but returned on 21st day of illness when muscle power also improved. Follow up NCS confirmed regeneration of nerves of all four limbs. So in any patient presenting with acute quadriparesis GBS should be in the differential diagnosis even if there is preserved or exaggerated deep tendon reflexes.J Bangladesh Coll Phys Surg 2014; 32: 107-109

Acute disseminated encephalomyelitis in a child with chikungunya virus infection

An 8-year-old boy was admitted to the Pediatric ward with high fever and severe arthritis of 4 days duration. Chikungunya virus serology was positive. As he became afebrile one week after admission, he developed acute onset right sided hemiparesis and suffered from one episode of generalized tonic clonic seizure. The next day he developed flaccid quadriplegia, loss of all sensory modalities below the C5 dermatome and urinary retention. After 4 weeks of shock stage, spasticity appeared in all 4 limbs. Magnetic resonance imaging (MRI) brain and spine showed extensive areas of demyelination-suggestive of acute disseminated encephalomyelitis (ADEM). He was treated with intravenous methyl prednisolone for 5 days, followed by oral prednisolone for 6 weeks. He was left with gross neurodeficits, including confinement to a wheel chair, persistent sensory loss and need for bladder catheterization.

Hypokalemic respiratory paralysis due to distal renal tubular acidosis as the presenting manifestation of Sjögren's syndrome

Hypokalemic respiratory paralysis in Sjögren's syndrome (SS) with distal renal tubular acidosis (RTA) is very rare. In the literature, only five such cases have been reported. We report a 48-year-old lady, who presented with respiratory paralysis, bulbar weakness, and flaccid quadriplegia. She had severe hypokalemia (1.11 mEq/L), metabolic acidosis (pH = 7.09), hyperchloremia (120.8 mEq/L), and normal anion gap (11.2). An ammonium chloride test was consistent with the diagnosis of distal RTA. She recovered completely with potassium and alkali supplementations. Clinical features, positive Schirmer's test, and autoantibody screening were suggestive of diagnosis of SS. Hypokalemic periodic paralysis and respiratory involvement may occur as a first and rare complication of SS with distal RTA. Immunosuppressive therapy is not indicated except in the presence of systemic vasculitis. Potassium and alkali supplementations are the mainstay of the therapy. Patients with secondary hypokalemic periodic paralysis should be investigated for this possibility.

Quadriplegia Due to Potassium Depletion: A Case Report

Hypokalemia is a very frequent metabolic disorder seen in emergency medicine, paralysis related to depletion hypokalemia is rare. We present the case of a 22 years old male patient who presented with flaccid quadriplegia after vomiting. After potassium supplementation the patient completely recovered. We will explain through this case the pathogenesis of this paralysis and different pitfalls that must be avoided.

A Rare case of Guillain-Barré syndrome in pregnancy treated with plasma exchange

Guillain-Barre syndromé (GBS) is an autoimmune disorder. It is rare in pregnancy as there is a decrease in cell-mediated immunity. A case of 28-year-old pregnant woman who presented with acute flaccid quadriplegia suffering from GBS is discussed in this study. She was treated with plasma exchange in her immediate post-partum period. The management of GBS in pregnancy has been discussed.

Flaccid Quadriplegia Due to Thyrotoxic Myopathy

Acute flaccid paralysis is an important clinical problem in neurological critical care. After implementing life-supporting measures, it is imperative to identify the correct diagnosis to provide timely appropriate care. Thyrotoxicosis is a recognized cause of myopathy, but rarely of quadriplegia. Here, we report a case of hyperthyroidism with severe weakness. Case report and video demonstration of clinical examination. We describe a case of a 59-year-old woman with Grave's disease who presented to the hospital with progressive shortness of breath secondary to atrial fibrillation with rapid ventricular response. Following contrast administration, she had a pulseless electrical activity arrest from which she recovered without cognitive sequelae, but with flaccid quadriplegia, facial diplegia, and hypophonia. CK was mildly elevated and electrolytes were essentially normal. Nerve conduction studies and electromyography demonstrated features supporting an acute myopathy without evidence of neuromuscular junction conduction abnormality. Normalization of thyroid hormones resulted in slow, but steady improvement over months after which she regained ambulation. Acute flaccid quadriplegia can result from thyrotoxicosis. With normalization of thyroid function, recovery can be expected.

Thyrotoxic Periodic Paralysis: Diversity in America

BackgroundThyrotoxic periodic paralysis (TPP) is a potentially life-threatening complication of thyrotoxicosis, characterized by hypokalemia and muscle paralysis. ObjectiveTPP, although becoming more prevalent in western countries, is still often not recognized due to lack of familiarity and the subtlety of the thyrotoxic symptoms. Early recognition by emergency physicians can prevent potential mortality. Case ReportA 23-year-old Vietnamese male presented with a 2-h history of complete flaccid quadriplegia and chest tightness. Electrolyte studies revealed a critical potassium level of 1.4 mmol/L. The patient was admitted to the intensive care unit where further workup revealed thyrotoxicosis and the patient was subsequently diagnosed with TPP. ConclusionsTPP should always be considered as a differential in patients, especially in young males of Asian descent with lower-limb paralysis or weakness. We present this case, followed by a review of the literature.

Spinal cord infarction associated with use of phentermine

A 33-year-old African American woman was admitted with acute onset weakness involving all her extremities of 1-day duration. She experienced pain in her retrosternal and cervical regions, followed by acute onset of numbness and weakness of all her extremities with alteration in tone of her speech. Except for phentermine intake for obesity for the last 3 months, the rest of her medical history was unremarkable. Her vital signs were stable, but the forced vital capacity (FVC) was 0.6 l, negative inspiratory force (NIF) was -45 cmH20, body mass index (BMI) was 38.5 kg/m . Cranial nerve examination was normal except for hypo phonic voice. She had flaccid quadriplegia, diffuse areflexia and mute plantar response. Sensation was decreased to all modalities from C3 (cervical) down with loss of bladder and rectal sphincter control. She had elevation of her serum glucose (221 mg/dl), hemoglobin A1C (9.3 %). Her total cholesterol was 157 mg/dl with low-density lipoprotein levels 97 mg/dl. Basic lab works including CSF examination were normal. Transthoracic and transesophageal echocardiography were normal. Magnetic resonance imaging (MRI) of the brain and spine with and without contrast including diffusionweighted imaging demonstrated acute infarction extending from lower left medulla to T3 (thoracic) vertebral body level predominantly involving the anterior cord with corresponding T2-weighted hyper intense signal abnormality (Fig. 1). Computed tomography (CT) of the chest, abdomen and pelvis with contrast and conventional angiogram of the brain and spine failed to reveal abnormalities of the aorta, extra cranial and intracranial vasculature.

A RARE CAUSE OF A COMMON PRESENTATION

A 26-year-old man presented with a 3 week history of progressive limb weakness and parasthesia following symptoms suggestive of a viral illness. He also developed diplopia, dysarthria and dysphagia. He...

Chronic subdural haematoma revealed by quadriparesis: A case report

Abstract Introduction A subdural haematoma is an accumulation of blood between the brain and its surrounding dural covering which acts as a space-occupying lesion causing increased intracranial pressure and cerebral compression. Chronic subdural haematomas (CSDH) differ from acute subdural haematomas in so far as occurring mainly in the elderly. A history of direct head trauma is absent in upto 50% of cases. Case report A 64-year old man, presented to the emergency centre with a sudden onset of right leg weakness. He initially denied any head injury but later recalled a minor head injury some weeks prior. Half an hour after presentation, his weakness progressed to a complete quadriparesis, while his sensory function remained intact and two hours after presentation he developed a complete flaccid quadriplegia. A CT scan was performed and revealed bilateral CSDH. Bilateral posterior and frontal burr holes were made under local anaesthesia, and 100ml of blood was evacuated. Clinical improvement was almost immediately noted. After two days, his neurologic examination was without any abnormality. Discussion CSDH is more common in the elderly. As reported here, clinical features may be delayed after the causal head injury (often weeks), and the trauma may even be forgotten. Cases manifesting bilateral haematomas are quite rare. The mechanism for quadriparesis is not fully understood. Motor deficit in the arms can be explained by direct compression or distortion of the cerebral hemispheres. This would not, however, account for motor weakness in the legs because the cortical areas responsible for the lower limbs would be relatively protected from the direct effect of compression. CSDH can present in many clinical scenarios, and may develop in the absence of a reported head injury. The diagnosis of CSDH should be considered in the differential when investigating cases of quadriplegia of uncertain aetiology, especially in the elderly population.

Involuntary Craniofacial Lingual Movements in Intensive Care-Acquired Quadriplegia

The syndrome of involuntary craniofacial lingual movements in the setting of acute intensive care-acquired quadriplegia (critical illness neuromyopathy) following sepsis-associated encephalopathy has not been previously described. We suggest a localization and treatment for this disabling condition. Three patients (2 female) from our center were quadriplegic from critical illness neuromyopathy when they developed involuntary craniofacial lingual movements following sepsis-associated encephalopathy. Extensive investigations failed to identify an etiology for the abnormal movements. Movements were of large amplitude, of moderate speed, and semi-rhythmic in the jaw, tongue, and palate, persistent and extremely bothersome to all patients. Injection with Botulinum toxin type A was very beneficial. Involuntary craniofacial lingual movements in the setting of flaccid quadriplegia following sepsis-associated encephalopathy are consistent with focal craniofacial brainstem myoclonus and constitutes a new syndrome. Botulinum toxin type A treatment maybe helpful in treatment.

Thyroid Storm and Reversible Thyrotoxic Cardiomyopathy After Ingestion of Seafood Stew Thought to Contain Marine Neurotoxin

Thyroid storm and severe decompensated thyrotoxicosis are life-threatening diseases, requiring immediate treatment in an intensive care unit. Thyrotoxic heart disease is common in patients with hyperthyroidism; thyrotoxic cardiomyopathy with poor left ventricular systolic function is rare. Here, we report a patient who presented with severe decompensated thyrotoxicosis and reversible thyrotoxic cardiomyopathy after ingestion of a mixed seafood stew thought to contain tetrodotoxin or a related marine neurotoxin. The patient was a 50-year-old woman who was seen at the emergency room of another hospital with altered mentality and flaccid quadriplegia after eating a mixed seafood stew. At the other hospital, she was diagnosed as having tetrodotoxin intoxication on the basis of her clinical presentation. The patient was placed on assisted ventilation and transferred to our hospital the following day because of persisting fever, tachycardia, and respiratory depression. Upon admission, thyroid storm was diagnosed on the basis of thyroid function tests (total triiodothyronine was 354 ng/dL, free thyroxine was 3.45 ng/dL, and thyroid-stimulating hormone was <0.01 mU/L) and clinical manifestations. On the third hospital day, she complained of chest discomfort and progressively worsening dyspnea. Echocardiography showed reduced left ventricular systolic function (left ventricular ejection fraction, 47%; left ventricular fractional shortening, 24.4%) with regional wall motion abnormality of anteroseptal wall and apex and diastolic dysfunction (grade 1, relaxation abnormality), suggesting thyrotoxic cardiomyopathy. She was treated with 10 mCi of radioactive iodine and methimazole, and her thyroid and cardiac function normalized. The patient reported here developed thyroid storm and cardiac decompensation shortly after a clinical event consistent with ingestion of marine neurotoxin. This adds to the list of stresses that have been associated with thyroid storm and emphasizes the need to fully evaluate patients who, on initial evaluation, appear to have only a single disorder.

Familial amyloid polyneuropathy: A clinico-pathologic study

In familial amyloid polyneuropathy (FAP), destruction of nerve fibres is related to accumulation of mutated transthyretin (mTTR) derived amyloid deposits (AD) in the endoneurium. Liver transplantation (LT), which removes the main source of mTTR, does not prevent deterioration of the clinical condition in all recipients. Material and methodsWe evaluated the distribution of AD in the central and peripheral nervous system in order to better understand the pathophysiology of FAP and the potential role of lesions of nerve blood vessels and of mTTR released by choroid plexuses (CP). Forty nerve biopsy specimens and 3 autopsy cases, including 7 patients who underwent liver transplantation, all from patients with symptomatic FAP and DNA mutation of the TTR gene, were included. ResultsPatients were ranged into three categories:Group 1:Patients with early neuropathic manifestations. Five patients carrying an amyloidogenic mutation closely followed to detect early neuropathic manifestations experienced paresthesiae with stockings temperature and pains deficit in two; uncertain changes in the others. Sensory nerve action potentials (SNAPs) were normal. Biopsy was performed to detect morphological changes as an indication for early therapeutic intervention. Only one patient had decreased MF and unmyelinated fiber (UF) density.Group 2:Patients with marked sensory-motor and dysautonomic polyneuropathy (35 patients). Non dissociated sensory loss prevailed in distal limbs, while sensory defect predominated on temperature and pain sensations in proximal limbs and anterior trunk. Weakness and amyotrophy predominated in distal lower limbs. Fiber loss massively predominated on UF.Group 3:Patients at terminal stage of the disease. All 3 patients had been bedbound for months with flaccid quadriplegia and non dissociated sensory deficit affecting all four limbs, up to proximal thighs, arms and anterior trunk along with severe autonomic dysfunction. There was virtually no surviving nerve fibers in distal nerves. Morphological changesAmyloid predominated around endoneurial capillaries in 37 patients, with occlusion/destruction of endoneurial capillaries in 15 nerves at late stages of the disease. Post-mortem examination showed amyloid in choroid plexuses and perivascular spaces in the brain and around blood vessels penetrating the endoneurium, following arachnoid and connective tissue septae. Destruction of endoneurial blood vessels is a late event in the natural course of FAP. Morphological findings were similar in patients who underwent liver transplantation and in those who did not. The distribution of amyloid in areas communicating with the subarachnoid space suggests that mutated TTR released in the CSF may move to the endoneurial fluid and accumulate in peripheral nerves, accounting for lack of efficacy of liver transplantation in some individuals.

Guillain-Barré Syndrome with Absent Brainstem Reflexes – a Report of Two Cases

Guillain-Barré syndrome, known for its diverse, atypical and heterogeneous range of presentations, can rarely present in an apparent comatose state with absent brainstem reflexes. Two patients presented in an unresponsive state with flaccid quadriplegia, total areflexia with no response to cephalic or peripheral painful stimuli. Pupils were mid-dilated with absent direct and consensual light reflex. All cranial nerve reflexes were absent. Preliminary laboratory investigations and complementary tests were normal. Cerebrospinal fluid evaluation showed albumincytological dissociation. Brain magnetic resonance imaging and electroencephalogram were normal. Both were diagnosed as fulminant Guillain-Barre syndrome. Despite an absence of brainstem reflexes neither patient fulfilled diagnostic criteria for brain death.

Fulminant Guillain–Barré syndrome after closed head injury: a potentially reversible cause of an ominous examination

Fulminant Guillain-Barré syndrome (GBS) is a rapidly progressive form of polyneuropathy in which patients demonstrate eventual flaccid quadriplegia and an absence of brainstem function. Most patients present after a mild upper respiratory or gastrointestinal illness and have nondiagnostic cerebral imaging studies. The authors present a case of fulminant GBS that developed in a 55-year-old alcoholic man 1 week after admission for a closed head injury. The details of this case and a discussion of GBS will be presented. This case provides evidence for combined central and peripheral nervous system involvement in severe cases of GBS. Recognition of fulminant GBS is important to prevent inappropriate declaration of brain death or withdrawal of support in the face of a potentially reversible process.

Birth-related spinal cord injuries: a multicentric review of nine cases

The objective of this study was to report a multicentric study of nine cases of children presenting with a birth-related spinal injury. The medical charts of nine patients identified by a questionnaire sent to the members of the French Society of Paediatric Orthopaedics (SOFOP) were reviewed. The pregnancy was uneventful in all cases. The fetal presentation was cephalic in three cases, a breech presentation in four cases, a face presentation in two cases. The lesion involved the cervical spine in six cases. Three patients presenting upper cervical injuries died before the age of 6 years. The six remaining patients experienced no neurological improvement. These rare conditions occur during difficult deliveries with abnormal presentations, the most common being a breech presentation with entrapment of the fetal head. In a child with hypotonia, flaccid quadriplegia or high thoracic paraplegia after a difficult delivery, a spinal cord injury must be suspected and plain radiographs and magnetic resonance imaging (MRI) must be performed.

Spinal cord injuries at birth: A multicenter review of nine cases

Objective. To report a multicenter study of nine cases of children presenting with a birth-related spinal injury.Methods. The medical charts of nine patients identified by a questionnaire sent to the members of the French Society of Pediatric Orthopedics (SOFOP) were reviewed.Results. The pregnancy was uneventful in all cases. The fetal presentation was cephalic in three cases, a breech presentation in four cases, and a face presentation in two cases. The lesion involved the cervical spine in six cases. Three patients presenting upper cervical injuries died before the age of six years. The six remaining patients experienced no neurological improvement.Conclusions. These rare conditions occur during difficult deliveries with abnormal presentations, the most common being a breech presentation with entrapment of the fetal head. In a child with hypotonia, flaccid quadriplegia or high thoracic paraplegia after a difficult delivery, a spinal cord injury must be suspected and plain radiographs and MRI must be performed.

Locked-in state due to Epstein–Barr virus primary infection in an immunocompetent patient

Sir: Guillain–Barre syndrome (GBS) can occasionally present with acute quadriparesis and abolition of brain stem reflexes [1]. We report a fulminant presentation of GBS mimicking a locked-in state during an Epstein–Barr virus (EBV) primary infection in an immunocompetent patient. A previously healthy 59-year-old woman was hospitalized because of weakness and dysaesthesia in the extremities for 24 h. One week before admission, she had had isolated fever for 72 h which had spontaneously resolved. At admission, the physical examination showed only a symmetric weakness of the legs (strength, 4/5), and preserved deep tendon reflexes. Laboratory tests showed an increase of aminotransferase plasma concentration (3N), no inflammatory syndrome, and a complete blood count normal without atypical leukocytes. The cerebrospinal fluid was normal. Magnetic resonance imaging (MRI) of the brain and the entire spine showed no abnormality. Over the next 24 h, the patient developed a flaccid quadriparesis, hyporeflexia, diplopia, difficulty in swallowing, and respiratory failure. She required intubation and mechanical ventilation. The electromyography (EMG) showed motor and sensory abnormalities in all four limbs consistent with a severe axonal polyneuropathy. A diagnosis of GBS was made and intravenous immunoglobulin (IVIg) (0.4 g/kg/day) was started. On the 4th hospital day, the patient presented full flaccid quadriplegia and absence of brain stem reflexes, in particular dilated pupils unresponsive to bright light, complete ophthalmoplegia, and no corneal reflex. An electroencephalogram (EEG) showed symmetrical 7–8 Hz activity responsive to visual and sound stimuli. Serologic tests for HIV, B and C hepatitis, Campylobacter jejuni and anti-ganglioside antibodies were negative. The results of serologic tests for EBV are shown in Table 1. EBV was not detected by PCR in CSF. After 5 days of IVIg, corticosteroids (1 mg/kg/day) were administrated for 4 weeks. The patient recovered progressively, with ocular motricity on day 9, head movement on day 15, and motricity of the arms beginning on day 30. She was extu-

Clinical and electrophysiological findings and long-term outcomes in paediatric patients with critical illness polyneuromyopathy

Neuromuscular weakness in paediatric patients with sepsis and multiple organ dysfunction is increasingly reported. However, many aspects of neuromuscular involvement in critically ill children are not completely understood. As more patients survive the critical illness, an understanding of the long-term outcomes of this condition is needed. To describe clinical and electrophysiological features and evaluate the long-term outcomes in critically ill paediatric patients with neuromuscular complications. A case series of five critically ill children was observed prospectively for a 1-month period. Selected clinical and laboratory parameters were evaluated. Electrophysiological studies were performed during the first week and then 1 month later in order to detect signs of critical illness polyneuromyopathy (CIPM). Patients with neuromuscular involvement completed a 1-year follow-up. Electrophysiological abnormalities were detected in two patients. Flaccid quadriplegia was a clinical presentation. Both children had electromyographic evidence of chronic partial denervation at follow-up, findings indicative of a preceding axonal neuropathy. Marked but incomplete recovery within 1 year after the onset of the disease occured in both patients. With a mild residual functional handicap the health-related quality of life was not significantly impaired (Barthel Index > 80). In both our patients with CIPM, the long-term clinical outcome is markedly better than we expected when electromyography in the 1-year follow-up demonstrated a persistent severe chronic partial denervation. These findings can have important implications for the management and rehabilitation of paediatric intensive care survivors.

Guillain‐Barré and Miller Fisher syndromes occurring with tumor necrosis factor α antagonist therapy

Diverse neurologic syndromes have been described in association with tumor necrosis factor alpha (TNFalpha) antagonist therapy for inflammatory arthritides and Crohn's disease. The objective of this study was to review the occurrence and clinical features of Guillain-Barré syndrome and its variant, the Miller Fisher syndrome, during TNFalpha antagonist therapy. The postmarketing database of the US Food and Drug Administration (FDA) was searched, following our experience with a patient with rheumatoid arthritis in whom the Miller Fisher syndrome variant of the Guillain-Barré syndrome developed while he was receiving infliximab therapy. Our index patient had a neurologic illness defined initially by ataxia and dysarthria, which fluctuated in relation to each subsequent infliximab infusion and, after 6 months, culminated in areflexic flaccid quadriplegia. In addition, 15 patients in whom Guillain-Barré syndrome developed following TNFalpha antagonist therapy were identified from the FDA database. Guillain-Barré syndrome developed following infliximab therapy in 9 patients, following etanercept therapy in 5 patients, and following adalimumab therapy in 1 patient. Among the 13 patients for whom followup data were available, 1 patient experienced no resolution, 9 patients had partial resolution, and 3 patients had complete resolution of Guillain-Barré syndrome following therapy. An association of Guillain-Barré syndrome with TNFalpha antagonist therapy is supported by the worsening of neurologic symptoms that occurred in our index patient following each infusion of infliximab, and by the temporal association of this syndrome with TNFalpha antagonist therapy in 15 other patients. An acute or subacute demyelinating polyneuropathy should be considered a potential adverse effect of TNFalpha antagonist therapy.