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Key Clinical Message: Clinicians should consider central venous thrombosis (CVT) as a differential diagnosis in young adolescents with persistent headaches. It is essential to assess for concurrent CVT and dural arteriovenous fistula (DAVF), particularly in those with a history of CVT. Abstract: Cerebral venous thrombosis (CVT) and dural arteriovenous fistula (DAVF) are uncommon vascular disorders with diverse clinical presentations. The coexistence of CVT and DAVF is a rare but important association that may impact the management and prognosis of affected patients. Prothrombotic conditions generally ranging from acquired to genetic, oral contraceptives, malignancy, puerperium, infection, and head injury are the common risk factors for cerebral venous thrombosis. Here, we present a case of 18 years males who developed recurrent cerebral thrombosis on the background of the presence of an arteriovenous fistula.
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The coronavirus disease 2019 (COVID-19) pandemic has affected the number of stroke activations, admission of patients with various types of strokes, the rate and timely administration of reperfusion therapy, and all types of time-based stroke-related quality assessment metrics. In this study, we describe the different types of strokes, different delays in seeking and completing treatment occurring during the second wave of the COVID-19 pandemic, and predictors of outcome at 3 months follow-up. Materials and methods: This is a single-centered prospective cross-sectional study carried out from May 2021 to November 2021, enrolling patients with stroke. Data collected were demographic characteristics, stroke types and their outcomes, and different types of prehospital delays. Results: A total of 64 participants were included in the study with a mean age of 60.25±15.31 years. Ischemic stroke was more common than hemorrhagic stroke. The median time of arrival to the emergency room of our center was 24 h. The most common cause of prehospital delay was found to be delays in arranging vehicles. The median duration of hospital stays [odds ratio (OR)=0.72, P<0.05] and baseline NIHSS (National Institute of Health Stroke Scale) score (OR=0.72, P<0.05) were found to be a predictor of good outcomes at 3 months follow-up on binary logistic regression. Conclusion: The factors that cause the delayed transfer to the hospital and onset of treatment should be addressed. Patient counseling about the likely prognosis can be done after evaluating the probable outcome based on the NIHSS score and median duration of hospital stay. Nevertheless, mechanisms should be developed to reduce the prehospital delay at the ground level as well as at the policy level.
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Painful tonic spasms initially described in association with multiple sclerosis are actually more common in patients with neuromyelitis optica spectrum disorder. Characterized by fierce pain and tonic posture of limbs, painful tonic spasms are common in patients during the recovery phase after the first episode of myelitis. A 68-year-old man presented with painful tonic spasm after 2 months of diagnosis of neuromyelitis optica spectrum disorder. Eventual use of eslicarbazepine resulted in significant control of spasms. Early recognition of painful tonic spasms and appropriate therapeutic medications can significantly decrease the impact it can have on the quality of life among neuromyelitis optica spectrum disorder patients.
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Anti-N-methyl D-aspartate (NMDA) receptor encephalitis is an autoimmune neurologic disorder that classically presents with psychiatric, neurologic, and autonomic symptoms, often with a viral prodrome. Case presentation: A 17-year-old female presented to the hospital with an 11-day history of fever, altered behavior, abnormal body movements, and altered sensorium. Upon examination, she was found to be febrile, tachycardic, and tachypneic, with a Glasgow Coma Scale score of 8. Discussion: The diagnosis of anti-NMDA receptor encephalitis is usually confirmed by the presence of anti-NMDA receptor antibodies in the cerebrospinal fluid. The first-line treatment options include steroids, intravenous immunoglobulin, and plasmapheresis, while second-line therapies such as rituximab and cyclophosphamide may be necessary for some patients. While most patients respond well to treatment, complications can arise, and as in this case, death can occur. Conclusion: New onset symptoms like alteration in behavior, abnormal body movement, altered sensorium, and psychiatric symptoms in a young female should raise suspicion of this disease. Immunotherapy is promising; however, anticipation and management of complication are essential in reducing mortality.
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Neurologic manifestations in primary Sjögren syndrome (SS) range in prevalence from 8 to 49%, and most of the studies suggest a prevalence of 20%. The incidence of SS patients developing movement disorders is about 2%. Case presentation: The authors herein report a case of a 40-year-old lady with MRI of the brain mimicking autoimmune encephalitis in SS who presented with chorea. Her MRI findings revealed T2 and FLAIR (fluid-attenuated inversion recovery) high signal intensity areas in bilateral middle cerebellar peduncles, dorsal pons, dorsal midbrain, hypothalami, and medial temporal lobes. Clinical discussion: There is still no evidence to support the definite use of MRI in characterizing the central nervous system involvement in primary SS, especially due to overlapping findings with age and cerebrovascular disease. Multiple areas of increased signal intensity in periventricular and subcortical white matter in FLAIR and T2-weighted image is commonly seen in primary SS patients. Conclusion: It is crucial to consider autoimmune diseases like SS as a cause of chorea in adults, even in those whose imaging findings are suggestive of autoimmune encephalitis.
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The type, quantity, and potency of the organophosphorus compound (OPC) taken determine the symptoms of OPC poisoning as well as their severity. The exact etiology for organophosphorus (OP) poisoning delay neuropathy regulating Wallerian degeneration is still unknown. Case Presentation: We report here a rare case of a 25-year-old lady with Wallerian degeneration in the brain found in an MRI in a patient after OPC ingestion. MRI of the brain, in our case, shows Wallerian degeneration of the corona radiata, internal capsule, and midbrain. Clinical Discussion: Some OPCs can lead to OP-induced delayed neuropathy, a form of delayed neurotoxicity in humans (OPIDN). The distal axonopathy's (in OPIDN) morphological pattern resembles Wallerian degeneration, which happens in vitro following nerve damage. Although delayed Wallerian degeneration from organophosphate poisoning often affects the peripheral nervous system, it can also affect the central nervous system. Rehabilitation therapy combined with appropriate nursing care has been demonstrated to improve the disease. Conclusion: Central nervous system involvement after OP poisoning is rare, and MRI of the brain and spinal cord can document evidence of Wallerian degeneration after OP poisoning.
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Stiff Person Syndrome (SPS), a progressive Central Nervous System disorder is accompanied by progressive muscle rigidity, hyperreflexia, and spasms mainly in truncal and proximal leg muscles mainly associated with autoimmune disorders. Here, we report a rare case of SPS in a middle-aged Nepalese man with uncontrolled diabetes mellitus and ketonuria.
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A rare case of chorea hyperglycemic basal ganglia syndrome in a 56-year-old woman who presented with left-sided hemichorea in the setting of uncontrolled, non-ketotic, type II diabetes mellitus is reported. Early blood glucose control could lead to complete resolution of symptoms. Despite an excellent prognosis, delayed recognition and management can lead to prolong disability due to movement disorder.
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Osmotic demyelination syndrome is a rare condition reported mainly in the case of rapid correction of hyponatremia, but it can occur even in the case of complicated diabetes mellitus either during rapid correction of hyperglycemia or anytime during the complicated diabetes mellitus. We report a case of complicated diabetes mellitus developing osmotic demyelination syndrome. The patient had presented with altered sensorium and seizure, which was initially diagnosed as hyperglycemia, but during his treatment, the magnetic resonance imaging of brain revealed central pontine myelinolysis. Our search on the causes of osmotic demyelination syndrome other than rapid correction of hyponatremia has revealed several other causes like autoimmune liver disease, Sjogren's syndrome and non-Hodgkin's lymphoma in addition to diabetes mellitus.
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Symptoms of Guillain-Barre Syndrome (GBS) may be mistaken for typical puerperal changes, delaying diagnosis. Surgery and anesthesia may be triggers for GBS with an overall increase in pro-inflammatory cytokines in the postpartum period. We report a unique case of GBS in the postpartum period who made a good recovery with supportive measures.
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ChAdOx1 nCoV-19 is an effective and well-tolerated coronavirus disease 2019 vaccine. However, rare cases of serious adverse events have been reported with it. We report a patient who did not have active or prior coronavirus disease 2019 infection, who developed Guillain-Barré syndrome 7 days following the first dose of ChAdOx1 nCoV-19 vaccination. He was treated with intravenous immunoglobulin, with stabilization of the disease. Proper monitoring and prompt reporting of such cases are required to ensure the safety of the vaccine.
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Organophosphate-induced delayed neuropathy, a central-distal axonopathy, passes through latent, progressive, static and improvement phases. During the improvement phase, the peripheral nerves regenerate unmasking the spinal cord lesion with myelopathic features. We report a case of a 16-year-old male who developed myelopathy 6 weeks following chlorpyrifos poisoning. He had a motor weakness of 4/5 in bilateral hips and 3/5 in bilateral knees and ankles. Spasticity and exaggerated reflexes with ankle clonus were present in the lower limbs. Sensory and the upper limb motor examinations were all normal. Pertinent blood, cerebrospinal fluid and nerve conduction tests were normal. Magnetic resonance imaging of the spine showed features of cord atrophy. Three months following physiotherapy, his power improved to 5/5 in bilateral knee and hip joints and 4/5 in bilateral ankles with spasticity. Organophosphate-induced delayed neuropathy can present as earlier as 6 weeks with myelopathy. Previous history of organophosphorous exposure is important in myelopathy or peripheral neuropathy.
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Introduction: Dengue is a common febrile illness caused by Dengue virus and spread by Aedes mosquitoes. The neurological complications like encephalopathy or encephalitis or immune-mediated neurological syndromes are uncommon though. Discrete neuroimaging findings in this setting are even rarer. We report a case of dengue encephalitis with uncommon MRI features in a young female. Case presentation: The patient presented with complains of fever, vomiting, weakness in all limbs and difficulty in speech. Neurological examination revealed bilateral horizontal gaze palsy with impaired oculo-cephalic reflex, bulbar dysarthria and quadriplegia with bilateral planters up-going. Laboratory reported anemia, thrombocytopenia and positive NS1 antigen while excluding other tropical and immunological diseases. Brain MRI revealed extensive thalamic involvement as unique "double-doughnut" sign along with lesions in brainstem. The patient received supportive treatment in intensive unit and was discharged following improvement in clinical condition and laboratory reports. Clinical discussion: Dengue can infect the central nervous system directly as encephalitis or can have neurological consequences following multi-organ dysfunction and shock as encephalopathy or post-infection immunological syndromes as Guillain-Barré Syndrome or cerebrovascular complications or dengue muscle dysfunction. The MRI appearance of "double-doughnut" sign points towards dengue encephalitis in appropriate setting. Conclusion: A high index of suspicion is required to make a diagnosis of dengue encephalitis. The "double-doughnut" sign in MRI sequences has the potential to become a diagnostic marker for dengue encephalitis.
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Neurotoxin-related optic neuritis (ON) after snake bite is uncommon. Here, we present a case of a 70-year-old female who developed bilateral painless loss of vision after she received treatment with anti-snake venom (ASV). She had only perception of light on assessment of visual acuity on admission which then improved drastically after administration of intravenous methylprednisolone (MP) after making the provisional diagnosis of ON on the basis of history and clinical findings of the patient. Imaging and visual-evoked potential could not be done initially, and they were done after the administration of intravenous MP which had normal findings. ASV, though being a lifesaving treatment, has been sometimes associated with ON.
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Coronavirus disease 2019 (COVID-19) has now spread widely after the outbreak since December 31, 2019. Guillain-Barré syndrome is an immunological postinfectious neuropathy, which has been reported to be a rare but possible complication COVID-19. We report a case of Guillain-Barré syndrome associated with COVID-19 in Nepal.
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Staphylococcus aureus is one of the common causes of infective endocarditis (IE). IE can present with various neurological complications such as stroke, brain abscess, and meningitis, the mortality rate can be very high in such cases.
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The diagnosis of rabies, a potentially fatal neuroinfectious disease, should be strongly considered in all patients who develop encephalitis following an infected animal bite even when they have received post-exposure prophylaxis. In the absence of confirmatory tests, typical magnetic resonance imaging findings help confirm the clinical diagnosis of rabies.
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Postoperative permanent hypoparathyroidism can exhibit extensive intracranial calcifications involving basal ganglia, thalamus, cerebellum, and cerebral cortex.
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We should suspect cerebral venous sinus thrombosis in patients with subacute onset progressive headaches with brain imaging evidence of localized cerebral edema with superimposed parenchymal, convexity subarachnoid as well as subdural hemorrhages.
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Apart from the usual differentials of transverse myelitis and cord compression, paraparetic GBS should be considered when sudden, flaccid paralysis of the lower limbs occurs, as prompt diagnosis and management can minimize sequel and unnecessary procedures. We do report a case wherein we managed a similar situation without the use of an immunomodulatory therapy.