Hyperreflexia

BACKGROUND: Guillain-Barré syndrome (GBS) is a rare entity with characteristic features, including progressive ascending paralysis. Patients typically present with progressive symmetrical weakness with areflexia in bilateral lower extremities, which can be confounded by psychiatric comorbidities. This case is unusual in that the patient had paradoxical hyperreflexia and normal CSF protein levels during her initial presentation, later confirmed to be GBS. CASE PRESENTATION: Here, we describe the case of a young female with bipolar disorder who presented to the hospital with complaints of week-long bilateral lower leg weakness that started abruptly about a month after an episode of multiple stools of bloody diarrhea...

INTRODUCTION: The standard of care for patients with infantile-onset Pompe disease (IOPD) is enzyme replacement therapy (ERT), which does not cross the blood brain barrier. While neuromuscular manifestations of IOPD are well-described, central nervous system (CNS) manifestations of this disorder are far less characterized. Here we describe severe CNS-related neurological manifestations including seizures and encephalopathy in six individuals with IOPD. METHOD: We identified six children with IOPD who developed CNS manifestations such as seizures and/or encephalopathy...

Intradural epidermoid cysts of the spine are rare congenital lesions. Their etiology is thought to stem from ectodermal remnants during embryonic development. They result in a diverse clinical presentation, often marked by an insidious onset and variable neurological deficits. Timely diagnosis is crucial for optimizing patient outcomes. We present the case of a 10-year-old male child presenting a six-month history of worsening back pain, intermittent leg weakness, and urinary incontinence. The physical examination revealed tenderness over the lower thoracic and lumbar spine, lower limb weakness, hyperreflexia, and sensory deficits...

Serotonin syndrome (SS) is an iatrogenic, drug-induced clinical syndrome caused by an increase in the intrasynaptic concentration of serotonin. Serotonin plays a significant role in the pathophysiology of migraines. Upregulation of 5-HT2A receptors is found in medication-overuse headache (MOH). Several migraine medications, both preventative and abortive drugs, act on serotonin receptors. We report two patients with chronic migraine who developed MOH. Besides headache, patients had frequent attacks of dizziness, restlessness, irritability, insomnia, excessive sweating, abdominal discomforts and tremors...

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism that manifests as painless flaccid paralysis. An East Asian man in his late 20s presented to the emergency department with an acute onset of quadriparesis associated with hypertonia and hyperreflexia. His initial symptoms and signs suggested involvement of the brain and spinal cord; however, MRI of the neuroaxis was normal. His serum potassium concentration was low, and thyroid test results were consistent with hyperthyroidism. The patient was diagnosed with TPP associated with Graves' disease and was treated with potassium supplementation, propranolol and methimazole...

OBJECTIVES: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is an early-onset ataxia characterized by cerebellar dysfunction, spasticity, and sensory-motor polyneuropathy due to variations in the SACS gene (13q11). To date, no studies have instrumentally assessed vestibular function in this condition. METHODS: We report a 36-year-old woman with diagnosis of ARSACS syndrome due to homozygous mutation (c.12232 C>T, p.Arg4078Ter) in the SACS gene...

Pantothenate kinase-associated neurodegeneration (or previously known as Hallervorden-Spatz syndrome) is a very rare disorder that typically manifests in a child with neurological signs such as gait difficulties, dysarthria, and hyperreflexia, associated potentially with psychiatric symptoms such as cognitive decline. It demonstrates on MRI the typical 'eye of the tiger' appearance, which is due to gliosis and accumulation of iron in the globi pallidi. Other differentials can mimic this appearance on MRI, it is therefore important to search for the involvement of other basal ganglia nuclei and the cerebral cortex, and also to consider the clinical and biological context...

Miller-Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome, characterized by ataxia, areflexia, ophthalmoplegia, and possible facial, swallowing and limb weakness alongside respiratory failure. Variations within MFS may include respiratory and limb weakness and Bickerstaff brainstem encephalitis (BBE), marked by altered consciousness, ataxia, ophthalmoparesis, and paradoxical hyperreflexia. MFS can emerge in both children and adults, often following bacterial or viral illness. While autoimmune-driven nerve damage occurs, most MFS patients recover within six months without specific treatment, with a low risk of lasting neurological deficits or relapses...

Serotonin syndrome (SS) describes a life-threatening clinical condition that can develop within hours or days after taking serotonergic medication(s). Medication adverse reactions, overdose, or drug interactions can cause this syndrome. Patients often present with symptoms of hyperthermia, muscle rigidity, hyperreflexia, and clonus. Symptoms range broadly in severity, often influenced by polypharmacy and age. In this report, SS was diagnosed in an elderly patient who presented with diffuse urticaria and exacerbated tremor...

BACKGROUND: Joint hyper-resistance is a common symptom in cerebral palsy (CP). It is assessed by rotating the joint of a relaxed patient. Joint rotations also occur when perturbing functional movements. Therefore, joint hyper-resistance might contribute to reactive balance impairments in CP. AIM: To investigate relationships between altered muscle responses to isolated joint rotations and perturbations of standing balance in children with CP. Methods & procedures: 20 children with CP participated in the study...

RATIONALE: A cervical pedicle screw (CPS) serves as an important anchor for cervical surgeries. Its placement requires the development of a highly safe and easy-to-handle method. Considering that the lateral end of the cortical bone of the spinal canal (LE point) is the most crucial for CPS placement, we devised a U-shaped wire capable of identifying LE points under direct vision and reliably confirming the site with C-arm lateral fluoroscopy. PATIENT CONCERNS: A 65-year-old male, who had been aware of numbness in both hands, mild finger dexterity disorder, and gait disturbance for half a year, visited our hospital due to the progression of his symptoms in the previous 2 months...

Spasticity is a hyperexcitability disorder that adversely impacts functional recovery and rehabilitative efforts after spinal cord injury (SCI). The loss of evoked rate-dependent depression (RDD) of the monosynaptic H-reflex is indicative of hyperreflexia, a physiological sign of spasticity. Given the intimate relationship between astrocytes and neurons, i.e., the tripartite synapse, we hypothesized that astrocytes might have a significant role in post-injury hyperreflexia and plasticity of neighboring neuronal synaptic dendritic spines...

Spasticity is a complex and multidimensional disorder that impacts nearly 75% of individuals with spinal cord injury (SCI) and currently lacks adequate treatment options. This sensorimotor condition is burdensome as hyperexcitability of reflex pathways result in exacerbated reflex responses, co-contractions of antagonistic muscles, and involuntary movements. Transcutaneous spinal cord stimulation (tSCS) has become a popular tool in the human SCI research field. The likeliness for this intervention to be successful as a noninvasive anti-spastic therapy after SCI is suggested by a mild and transitory improvement in spastic symptoms following a single stimulation session, but it remains to be determined if repeated tSCS over the course of weeks can produce more profound effects...

STUDY DESIGN: Delayed diagnosis of degenerative cervical myelopathy (DCM) is likely due to a combination of its subtle symptoms, incomplete neurological assessments by clinicians and a lack of public and professional awareness. Diagnostic criteria for DCM will likely facilitate earlier referral for definitive management. OBJECTIVES: This systematic review aims to determine (i) the diagnostic accuracy of various clinical signs and (ii) the association between clinical signs and disease severity in DCM? METHODS: A search was performed to identify studies on adult patients that evaluated the diagnostic accuracy of a clinical sign used for diagnosing DCM...

Clinical studies have shown that individuals with spinal cord injury (SCI) are particularly susceptible to infectious diseases, resulting in a syndrome called SCI-induced immunodeficiency syndrome (SCI-IDS), which is the leading cause of death after SCI. It is believed that SCI-IDS is associated with exaggerated activation of sympathetic preganglionic neurons (SPNs). After SCI, disruption of bulbospinal projections from the medulla oblongata C1 neurons to the SPNs results in the loss of sympathetic inhibitory modulation from the brain and brainstem and the occurrence of abnormally high levels of spinal sympathetic reflexes (SSR), named sympathetic hyperreflexia...

Cervical myelopathy is a well-described medulla spinalis syndrome characterized by sensory disorders, such as pain, numbness, or paresthesia in the limbs, and motor disorders, such as muscle weakness, gait difficulties, spasticity, or hyperreflexia. If left untreated, cervical myelopathy can significantly affect the quality of life of patients, while in severe cases, it can cause disability or even quadriplegia. Cervical myelopathy is the final stage of spinal cord insult and can result from transgene dysplasias of the spinal cord, and acute or chronic injuries...

OBJECTIVES: To observe the effect of electroacupuncture (EA) on urodynamics and Raf/MEK/ERK signaling pathway in spine cord tissue of rats after suprasacral spinal cord injury (SSCI), so as to explore its possible mechanism in improving bladder function in rats with detrusor hyperreflexia after SSCI. METHODS: Female SD rats were randomly divided into blank, sham operation, model, EA and EA+PD98059 groups, with 12 rats in each group. Thorax (T) 10 spinal cord transection was performed by surgery...

Spasticity is a chronic neurological complication associated with spinal cord injury (SCI), characterized by increased muscle tone and stiffness. A physiological sign of spasticity is hyperreflexia, evident by the loss of evoked rate-dependent depression (RDD) in the monosynaptic spinal circuit, i.e., H-reflex. Although previous work has shown that SCI-induced astrogliosis contributes to hyperexcitability disorders, including neuropathic pain and spasticity, it is unclear how reactive astrocytes can modulate synaptic transmission within the injured spinal cord...

Sclerosing meningiomas (SMs) represent a rare histological variant of meningiomas, first described in 1989 as invasive bulking masses of whorling collagen bundles with a minimum percentage of meningothelia-resembling cells, and they are often misdiagnosed. The literature reports only 30 cases of SMs, with only two of them being intramedullary. The authors present the case of a patient with a cervical intramedullary SM who presented with gait disturbances, sensory deficits, weakness in four extremities, and hyperreflexia...

Tetanus toxin (TeNT) is a protein toxin produced by Clostridium tetani bacteria, which causes hyperreflexia and rhabdomyolysis by spastic paralysis. Like botulinum neurotoxin, TeNT comprises a heavy chain (HC) and a light chain (LC) linked via an interchain disulfide bond, which include the following three functional domains: a receptor-binding domain (Hc), a translocation domain (HN), and a catalytic domain (LC). Herein, we produced and characterized three functional domains of TeNT and three types of TeNT-derived L-HN fragments (TL-HN, TL-GS-HN and TL-2A-HN), which contained L and HN domains but lacked the Hc domain...