{"title":"Muscle-Specific Tyrosine Kinase-Associated Myasthenia Gravis: A Neuromuscular Surprise.","authors":"Hassam Ali, Rahul Pamarthy, Nayab Ahsan, WashmaAwan, Shiza Sarfraz","doi":"10.1155/2021/1326442","DOIUrl":"https://doi.org/10.1155/2021/1326442","url":null,"abstract":"<p><p>Myasthenia gravis is a neuromuscular autoimmune disease that results in skeletal muscle weakness that worsens after periods of activity and improves after rest. Myasthenia gravis means \"grave (serious), muscle weakness.\" Although not completely curable, it can be managed well with a relatively high quality of life and expectancy. In myasthenia gravis, antibodies against the acetylcholine receptors at the neuromuscular junction interfere with regular muscular contraction. Although most commonly caused by antibodies to the acetylcholine receptor, antibodies against MuSK (muscle-specific kinase) protein can also weaken transmission at the neuromuscular junction. Muscle-specific tyrosine kinase myasthenia gravis (MuSK-Ab MG) is a rare subtype of myasthenia gravis with distinct pathogenesis and unique clinical features. Diagnosis can be challenging due to its atypical presentation as compared to seropositive myasthenia gravis. It responds inconsistently to steroids, but plasma exchange and immunosuppressive therapies have shown promising results. We report a case of a 49-year-old female who presented with acute hypoxic respiratory failure. Our patient experienced progressive, undiagnosed MuSK-Ab MG for years without a diagnosis.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"1326442"},"PeriodicalIF":0.9,"publicationDate":"2021-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8727168/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39668881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yao Christian Hugues Dokponou, Mamoune El Mostarchid, Housni Abderrahmane, Niamien Patrice Koffi, Miloudi Gazzaz, Brahim El Mostarchid
{"title":"Spontaneous Recovery of Penetrating Cervical Spinal Cord Injury with Physiotherapeutic Treatment: Case Report and Review of the Literature.","authors":"Yao Christian Hugues Dokponou, Mamoune El Mostarchid, Housni Abderrahmane, Niamien Patrice Koffi, Miloudi Gazzaz, Brahim El Mostarchid","doi":"10.1155/2021/3741461","DOIUrl":"https://doi.org/10.1155/2021/3741461","url":null,"abstract":"<p><p>Stab wounds to the cervical spine are less common than injuries from road accidents, sports injuries, and falls. The presence of vital, vascular, neural, respiratory, and digestive structures in the neck region mean that this kind of spinal injury is generally critical, and its management is a challenge. We report a unique case of a previously healthy 17-year-old adolescent admitted for quadriplegia secondary to a stab wound to the cervical spine at the C4C5 level. There was no surgical indication. The patient underwent physiotherapy. He showed spontaneous neurological improvement two weeks later and was able to sit on his own and to walk about three months of physical rehabilitation.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"3741461"},"PeriodicalIF":0.9,"publicationDate":"2021-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8720595/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39788034","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
James Liu, Yue Ding, Sandra Camelo-Piragua, James Richardson
{"title":"Vasculitic Tibial Mononeuropathy Associated with Inherited Immune Dysregulation: A Review of Tibial Mononeuropathies with Electrodiagnostic Considerations.","authors":"James Liu, Yue Ding, Sandra Camelo-Piragua, James Richardson","doi":"10.1155/2021/7161757","DOIUrl":"https://doi.org/10.1155/2021/7161757","url":null,"abstract":"<p><p>Compressive tibial mononeuropathies are uncommon and can be caused by conditions including posterior compartment syndrome, soleal sling syndrome, and tarsal tunnel syndrome. Therefore, it is critical to consider noncompressive etiologies when a tibial mononeuropathy is suspected. This is a patient with a history of rare inherited immune dysregulation that presented to the electrodiagnostic laboratory with severe neuropathic pain in the right foot associated with plantarflexion weakness, concerning for a tibial mononeuropathy. However, the patient's clinical presentation and results on electrodiagnostic testing were not consistent with any of the above entities. Therefore, noncompressive etiologies of tibial mononeuropathies such as vasculitis had to be considered. The patient subsequently underwent sural nerve biopsy which confirmed small-vessel vasculitis as the cause of the tibial mononeuropathy. She was then started on appropriate immunosuppressive treatment which resulted in significant pain relief and was discharged home. This case highlights the importance of considering noncompressive causes of tibial nerve injury. Compressive and vasculitic tibial mononeuropathies along with their electrodiagnostic considerations are reviewed. Furthermore, this case highlights the critical role of the electromyographer and ability to maximize the impact on patient care through a solid foundation in anatomy, pathophysiology, and electrodiagnosis blended with clinical acumen.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"7161757"},"PeriodicalIF":0.9,"publicationDate":"2021-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8604612/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39897529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Samantha Novotny, Kenneth Serrano, Danielle Bazer, Louis Manganas
{"title":"Multiple Cranial Nerve Palsies in a Pediatric Case of Lemierre's Syndrome due to <i>Streptococcus viridans</i>.","authors":"Samantha Novotny, Kenneth Serrano, Danielle Bazer, Louis Manganas","doi":"10.1155/2021/4455789","DOIUrl":"https://doi.org/10.1155/2021/4455789","url":null,"abstract":"<p><strong>Background: </strong>Lemierre's syndrome is a rare condition of internal jugular vein thrombosis following oropharyngeal infection. While it usually results from <i>Fusobacterium necrophorum</i> infection, atypical cases associated with other pathogens have been reported.</p><p><strong>Objective: </strong>To describe a unique case of pediatric Lemierre's syndrome with <i>Streptococcus viridans</i> infection resulting in cavernous sinus thrombosis and oculomotor, trochlear, and abducens nerve palsies. <i>Case Report</i>. A 14-year-old female initially presented after six days of fever, myalgias, and sore throat and was admitted for hyperbilirubinemia and acute kidney injury. She developed a fixed, dilated pupil with complete ophthalmoplegia, ptosis, and severe pain. Imaging revealed retromandibular space abscess, external and internal jugular vein thrombosis, cavernous sinus thrombosis, internal carotid artery stenosis, pulmonary embolism, and bilateral pneumonia. She was diagnosed with Lemierre's syndrome with cultures positive for <i>Streptococcus viridans</i> and treated with a combination of antibiotics and anticoagulation. <i>Conclusion and Relevance</i>. Both antibiotics and anticoagulation were effective management for this Lemierre's syndrome patient with cavernous sinus thrombosis. Early diagnosis and treatment of Lemierre's syndrome is essential. A multidisciplinary treatment team is beneficial for managing the sequelae of this condition.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"4455789"},"PeriodicalIF":0.9,"publicationDate":"2021-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8564190/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39686873","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Paraplegia after Hypotension with Pneumothorax Episode during Thoracic Spine Tumor Surgery.","authors":"Zhao-Quan Liu, Jen-Hsien Liao, Chih-Ju Chang","doi":"10.1155/2021/2232769","DOIUrl":"https://doi.org/10.1155/2021/2232769","url":null,"abstract":"<p><p>Paraplegia after spine surgery is a catastrophic complication. Here, we present a patient who, following laminectomy and fusion for decompression of metastatic tumor, developed paraplegia. We tried to find out the possible reason for the paraplegia. Due to prolonged hypotension during operation and new onset of pneumothorax, we think that intraoperative prolonged hypotension leads to the spinal cord ischemia which may cause neurological deterioration of paraplegia. Maintaining hemodynamic stability during spinal surgery is very important.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"2232769"},"PeriodicalIF":0.9,"publicationDate":"2021-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8557050/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39849159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Joshua Santucci, Naresh Mullaguri, Anusha Battineni, Raviteja R Guddeti, Christopher R Newey
{"title":"Paradoxical and Retrograde Air Embolism from Pressurized Peripheral Bolus.","authors":"Joshua Santucci, Naresh Mullaguri, Anusha Battineni, Raviteja R Guddeti, Christopher R Newey","doi":"10.1155/2021/1063264","DOIUrl":"https://doi.org/10.1155/2021/1063264","url":null,"abstract":"<p><strong>Introduction: </strong>Cerebral air embolism is a rare, yet serious neurological occurrence with unclear incidence and prevalence. Here, we present a case of fatal cerebral arterial and venous cerebral gas embolism in a patient with infective endocarditis and known large right-to-left shunt and severe tricuspid regurgitation following pressurized fluid bolus administration. <i>Case Presentation</i>. A 32-year-old female was admitted to the medical intensive care unit from a long-term acute care facility with acute on chronic respiratory failure. Her medical history was significant for intravenous heroin and cocaine abuse, methicillin-sensitive <i>Staphylococcus aureus</i> tricuspid valve infective endocarditis on vancomycin, patent foramen ovale, septic pulmonary embolism with cavitation, tracheostomy with chronic ventilator dependence, multifocal cerebral infarction, hepatitis C, nephrolithiasis, anxiety, and depression. After intravenous fluid administration, she became unresponsive with roving gaze, sluggish pupils, and hypotensive requiring vasopressors. CT of the brain showed diffuse arterial and venous cerebral air embolism secondary to accidental air administration from fluid bolus. Magnetic resonance imaging of the brain showed diffuse global anoxic injury and flattening of the globe at the optic nerve insertion. Given poor prognosis, her family chose comfort measures and she died.</p><p><strong>Conclusions: </strong>Fatal cerebral air embolism can occur through peripheral intravenous routes when the lines are inadequately primed and fluids administered with pressure. Caution must be exercised in patients with right-to-left shunting as air may gain access to systemic circulation.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"1063264"},"PeriodicalIF":0.9,"publicationDate":"2021-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8510818/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39519511","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Familial Multiple Sclerosis in a Mother and Son Pair: A Sri Lankan and a South Asian First.","authors":"Ishani Rajapakshe, Bimsara Senanayake","doi":"10.1155/2021/1172870","DOIUrl":"https://doi.org/10.1155/2021/1172870","url":null,"abstract":"<p><p>Multiple sclerosis (MS) is an immune-mediated demyelinating disorder involving the central nervous system (CNS). It is common amongst young females. Although the exact cause of MS is yet unknown, viral infections such as EBV, environmental factors, and autoimmune and genetic mechanisms involving HLA-DRB1 loci are implicated. Familial MS is reported from some geographic locations and ethnic groups but is thought to be rare in Asia. In this paper, we present both a Sri Lankan mother and her son, with clinically definite MS conforming to McDonald's 2017 clinical and MAGNIMS 2016 radiological criteria. Both had oligoclonal bands in their CSF (OCB-IEF) with no serum bands indicating intrathecal production and were negative for AQP4 and MOG IgG serology. Familial MS is more common among siblings, with sister-sister relationship having the highest rate. The lowest relation was amongst father-son and mother-son pairs. Amongst siblings, the risk of MS is between 3.5% and 4.7%. Inherited factors rather than common environmental exposure influence susceptibility in such cases. To the best of our knowledge, MS occurring in a mother-son pair has not been reported before either from Sri Lanka or South Asia.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"1172870"},"PeriodicalIF":0.9,"publicationDate":"2021-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8486554/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39482683","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carlo Fornaini, Zhao Meng, Elisabetta Merigo, Jean-Paul Rocca
{"title":"\"At-Home\" Photobiomodulation: A New Approach for Bell's Palsy Treatment.","authors":"Carlo Fornaini, Zhao Meng, Elisabetta Merigo, Jean-Paul Rocca","doi":"10.1155/2021/5043458","DOIUrl":"https://doi.org/10.1155/2021/5043458","url":null,"abstract":"<p><strong>Objective: </strong>This report is the first one to describe the possibility to use \"self-administered\" photobiomodulation (PBM) for Bell's palsy (BP) treatment.</p><p><strong>Background: </strong>BP is a peripheral disorder of the facial nerve causing sudden paralysis of unilateral facial muscles, and PBM has been successfully suggested for its treatment without any side effect. This is the first case report where a laser device was successfully used at home by the patient herself to treat BP opening new perspectives on the therapy of this disease.</p><p><strong>Methods: </strong>This report describes the \"at-home PBM\" treatment performed on a 15-year-old girl who presented BP consisting of acute pain on the right side of her face, difficulty in biting and dripping saliva from the right side of her lips. The treatment was performed twice a day by cutaneous applications, each of 15 minutes (total fluence 48 J/cm<sup>2</sup>) in an area corresponding to the parotid gland by a device emitting at 808 nm at 250 mW output power.</p><p><strong>Results: </strong>Two weeks after PBM treatment, performed at home twice a day by the patient herself without any kind of pharmacological therapy, the complete disappearing of the disease was noticed with no side effects.</p><p><strong>Conclusion: </strong>With the limitations due to a single case report and with the need of further clinical trials to confirm it, \"at-home PBM\" seems to represent a good and safe approach to the treatment of BP.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"5043458"},"PeriodicalIF":0.9,"publicationDate":"2021-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8455211/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39443836","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mick B Reedy, Yanping Wang, Brad R Beinlich, William Nicholas Rose
{"title":"Successful Treatment of Incomplete Susac Syndrome with Simultaneous Corticosteroids and Plasmapheresis Followed by Rituximab.","authors":"Mick B Reedy, Yanping Wang, Brad R Beinlich, William Nicholas Rose","doi":"10.1155/2021/5591559","DOIUrl":"https://doi.org/10.1155/2021/5591559","url":null,"abstract":"<p><p>We present a case report of a patient with incomplete Susac syndrome. He had cognitive impairment, corpus callosum lesions, and vestibulocochlear dysfunction on brainstem auditory evoked responses. He was treated with methylprednisolone and plasmapheresis, improved, and then, also received rituximab. His improvement has been lasting as of this writing. This case shares our experience with a successful treatment of this rare condition that is incompletely understood and lacks well-established treatment guidelines.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"5591559"},"PeriodicalIF":0.9,"publicationDate":"2021-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8457979/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39475406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Recurrent Hodgkin's Disease Presenting as a Clinically Isolated Cavernous Sinus Syndrome.","authors":"Aayesha Jalaluddin Soni, Edward Bernard Lee-Pan","doi":"10.1155/2021/3946231","DOIUrl":"https://doi.org/10.1155/2021/3946231","url":null,"abstract":"<p><strong>Background: </strong>Hodgkin's disease involving the central nervous system is uncommon and is usually seen in patients with uncontrolled systemic disease or those who have had multiple episodes of recurrent disease. Common symptoms of intracranial Hodgkin's disease are motor and/or sensory deficits, headache, papilloedema, coma, and seizures. The rarity of Hodgkin's disease presenting with intracranial involvement is marked, but patients presenting with cavernous sinus syndrome is even rarer. Despite its rarity, the presence of a cavernous sinus syndrome in a patient with a known history of Hodgkin's disease warrants full utilization of modern diagnostic techniques in terms of investigation. <i>Case Presentation</i>. A 34-year-old woman, known with previous Hodgkin's lymphoma and now in remission for the past 7 years, presented with signs and symptoms suggestive of a left cavernous sinus syndrome. She was otherwise systemically well with no other complaints. Extensive investigations revealed no obvious cause for the cavernous sinus syndrome. A CT chest revealed subclinical axillary lymphadenopathy, which on excisional biopsy confirmed recurrent Hodgkin's disease. The patient's sole clinical presentation of her recurrent disease was the cavernous sinus syndrome, with no other clinically obvious systemic signs or symptoms to suggest a relapse. This was treated with steroids, and clinical improvement was noted; she was referred to oncology for extensive chemotherapy.</p><p><strong>Conclusions: </strong>Whilst there is a paucity of literature around this topic, it must be remembered by the clinician that cavernous sinus syndrome may be the sole clinical presentation of recurrent Hodgkin's disease. Even though it is reported that lymphomatous involvement in the brain usually occurs late in the disease process, this case is evidence that cavernous sinus syndrome may precede other systemic signs and symptoms. Recognising this possibility will ensure a rigorous search for Hodgkin's disease, an early and effective diagnosis, and a better prognosis in affected patients.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":" ","pages":"3946231"},"PeriodicalIF":0.9,"publicationDate":"2021-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8369195/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39329111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}