Omar Alhaj Omar, Norma J Diel, Stefan T Gerner, Anna Mück, Hagen B Huttner, Heidrun H Krämer-Best
{"title":"Efgartigimod as Rescue Medication in a Patient with Therapy-Refractory Myasthenic Crisis.","authors":"Omar Alhaj Omar, Norma J Diel, Stefan T Gerner, Anna Mück, Hagen B Huttner, Heidrun H Krämer-Best","doi":"10.1155/2024/9455237","DOIUrl":"https://doi.org/10.1155/2024/9455237","url":null,"abstract":"<p><p>Myasthenic crises (MC) are potentially life-threatening acute exacerbations of myasthenia gravis (MG) characterized by profound muscle weakness, bulbar symptoms, and potential for respiratory failure. Intravenous immunoglobulins (IVIG) and plasma exchange (PLEX) are conventional treatments for myasthenic exacerbations. Recently, new therapeutic options for generalized acetylcholine-receptor antibody positive (AchR+) MG were approved as an add-on therapy. They mainly consist of complement C5 inhibitors such as eculizumab and ravulizumab and neonatal Fc receptor antagonists such as efgartigimod with the approval of more options pending, e.g., zilucoplan and rozanolixizumab. More therapeutic options are in the pipeline. Although the data show a quick and reliable treatment response, these medications have not been studied for the therapy of myasthenic crisis. We present the case of a 57-year-old male with his first episode of generalized myasthenia gravis (MG) and positive acetylcholine-receptor antibodies (AchR+) who was transferred to our neurological intensive care unit with worsening generalized weakness, dysphagia, and respiratory distress. The crisis was triggered by pneumonia due to dysphagia. He was diagnosed with myasthenic crisis and treated with intravenous pyridostigmine, plasmapheresis (PLEX), and continued prednisone. Initial improvement was followed by deterioration, requiring readmission and additional PLEX. After a further decline, efgartigimod was administered, leading to significant improvement within 48 hours, as evidenced by reduced MG-ADL and QMG scores. The patient continued to improve and was stable enough for transfer to a rehabilitation facility. This case illustrates the potential of efgartigimod as a novel treatment for refractory myasthenic crises.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2024 ","pages":"9455237"},"PeriodicalIF":0.9,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11211016/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141466409","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":"Chronic Inflammatory Demyelinating Polyradiculoneuropathy with Diplopia Caused by an Alternative Coronavirus Disease 2019 Vaccine.","authors":"Satoshi Saito, Mutsumi Iijima, Misa Seki, Ayato Shimomura, Kazuo Kitagawa","doi":"10.1155/2024/8584482","DOIUrl":"10.1155/2024/8584482","url":null,"abstract":"<p><p>The etiology of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) remains elusive and is believed to involve multiple contributing factors. There have been cases linking CIDP to the coronavirus disease 2019 (COVID-19) mRNA vaccine. However, there are no documented instances following alternative vaccines. We report a case of a 48-year-old woman, previously vaccinated with Pfizer-BioNTech's COVID-19 vaccine (BNT162b2), who subsequently received the Moderna mRNA-1273 vaccine. Within 2 days postvaccination, she developed diplopia and numbness in the lower limbs' distal extremities. Cerebrospinal fluid analysis exhibited protein-cell dissociation, while F-wave studies showed demyelinating activity in the bilateral tibial nerves. Given the disease's progressive nature, the patient was presumed to have CIDP and commenced steroid pulse therapy and intravenous immunoglobulin therapy. The onset of CIDP may be associated with variations in mRNA sequences and vaccine constituents.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2024 ","pages":"8584482"},"PeriodicalIF":0.9,"publicationDate":"2024-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11208806/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141466408","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":"Syncope vs. Seizure: Ictal Bradycardia and Ictal Asystole.","authors":"Sumika Ouchida, Kaitlyn Parratt, Armin Nikpour, Greg Fairbrother","doi":"10.1155/2024/1299282","DOIUrl":"10.1155/2024/1299282","url":null,"abstract":"<p><strong>Background: </strong>Ictal arrhythmia is a rare condition that causes arrhythmic manifestations induced by epileptic seizures, including asystole or bradycardia. Ictal asystole (IA) is a very rare condition found in patients undergoing video-encephalography (EEG) monitoring. It is often related to temporal lobe epilepsy and can cause syncope, which can lead to injury or even death. <i>Case Presentation</i>. Two patients with epilepsy showed symptoms of syncope. Both patients underwent 4-day ambulatory EEG tests and were diagnosed with IA. Following the tests, the patients were implanted with a permanent pacemaker, and one of them underwent a temporal lobectomy. As a result of these procedures, the patients experienced a reduction in episodes of symptomatic syncope.</p><p><strong>Conclusion: </strong>Patients with ictal asystole and symptomatic ictal bradycardia are at increased risk of falls due to seizures. Although there are no specific guidelines for managing this condition, antiseizure medications, epilepsy surgery, and cardiac pacemaker implantation have been effective treatments.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2024 ","pages":"1299282"},"PeriodicalIF":0.9,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11090674/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140916014","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}
Sakari Kaasalainen, Harri Arikka, Mika H Martikainen, Valtteri Kaasinen
{"title":"Novel SLC18A2 Variant in Infantile Dystonia-Parkinsonism Type 2.","authors":"Sakari Kaasalainen, Harri Arikka, Mika H Martikainen, Valtteri Kaasinen","doi":"10.1155/2024/4767647","DOIUrl":"10.1155/2024/4767647","url":null,"abstract":"<p><p>Infantile dystonia-parkinsonism type 2 (PKDYS2) is a rare inherited autosomal recessive movement disorder with onset in infancy. The disease is associated with a mutation in the solute carrier family 18 member A2 gene (<i>SLC18A2</i>). There are reports of trials with dopaminergic drugs and the condition of patients given levodopa almost always worsens and dopamine agonists give varying degrees of benefit to some. Here, we report a PKDYS2 patient with a new variant in the <i>SLC18A2</i> gene who underwent multiple trials of pharmacotherapy. The abnormalities in development and neurological examination of the case were first noted at the age of 2 months, and after a series of treatment attempts (e.g., with antiepileptics) and diagnostic procedures, the diagnosis of PKDYS2 was determined when whole exome sequencing (WES) at age 6, revealed a homozygous pathologic variant NM_003054.4:c.1107dup, p.(Val370Serfs<sup><i>∗</i></sup>91) in the <i>SLC18A2</i> gene. The patient then received treatment with multiple dopaminergic drugs (e.g., levodopa, pramipexole, and methylphenidate). The patient with PKDYS2 harbored a new variant in <i>SLC18A2.</i> The phenotype of the patient resembles that of some previously reported patients with PKDYS2. The patient received minor benefits from certain dopaminergic drugs, such as pramipexole, but side effects led to the discontinuation of tested medications.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2024 ","pages":"4767647"},"PeriodicalIF":0.9,"publicationDate":"2024-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11074866/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140875941","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}
Satoshi Takahashi, Masahiro Katsumata, Hirotsugu Nogawa, Kento Takahara, Jin Nakahara, Masahiro Toda
{"title":"A Case of Symptomatic Intraluminal Internal Carotid Artery Thrombus in a Patient with Essential Thrombocythemia Surgically Treated by CEA.","authors":"Satoshi Takahashi, Masahiro Katsumata, Hirotsugu Nogawa, Kento Takahara, Jin Nakahara, Masahiro Toda","doi":"10.1155/2023/9152009","DOIUrl":"10.1155/2023/9152009","url":null,"abstract":"<p><p>We report a patient with a symptomatic intraluminal internal carotid artery thrombus clinically revealed by cerebral infarction. In the preoperative evaluation, it was revealed that essential thrombocythemia existed in the background. Therefore, medical treatment with antithrombotic agents in conjunction with hydroxycarbamide for essential thrombocythemia was initiated, but the thrombus was not dissolved by three weeks. At this time, the patient underwent carotid endarterectomy, which removed the thrombus completely with its adjacent plaque without any perioperative stroke. The possibility of essential thrombocythemia may also be kept in mind when an increased platelet count is observed in patients with internal carotid artery thrombus. It is a reasonable option to precede medical treatment, including anticoagulant therapy, by setting the time limit for surgical intervention in such a disease state.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2023 ","pages":"9152009"},"PeriodicalIF":0.9,"publicationDate":"2023-11-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10691885/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138476843","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}
Issa Ibrahim Assoumane, Nicaise Kpègnon Agada, Rabiou Maman Sani, Aminath Kélani
{"title":"Penetrating Head Injury by a Hit of Rake in a Child: A Case Report and Literature Review.","authors":"Issa Ibrahim Assoumane, Nicaise Kpègnon Agada, Rabiou Maman Sani, Aminath Kélani","doi":"10.1155/2023/9921985","DOIUrl":"https://doi.org/10.1155/2023/9921985","url":null,"abstract":"<p><strong>Background: </strong>A penetrating head injury (PHI) refers to a situation where a projectile has breached the cranium but does not exit it. It constitutes about 0.4% of all head injuries. Several nonmissile materials inserting the skull have been reported. But to our knowledge, never before has any case of PHI caused by a hit of rake been reported. We report a first case of PHI caused by a rake in a child; then, we relate our experience with its management and discuss the relevant literature. <i>Cases Description</i>. A 5-year-old boy has been admitted with a rake embedded in his head. That occurred during a violent play with a neighbor. At presentation, the child was alert; there was no neurological deficit. The rake was embedded in the parietal regions on each side of the midline. The head Computed Tomography (CT) scan performed showed a biparietal hyperdensity from either side of the midline with a metal artifact. In the operating room, after a transversal incision joining the 2 tips of the object, we performed successively bone flaps; object extraction; debridement; duraplasty; and closing. The outcome was uneventful.</p><p><strong>Conclusion: </strong>This is the first case of PHI by a rake. The surgical management constitutes the main challenging point.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2023 ","pages":"9921985"},"PeriodicalIF":0.9,"publicationDate":"2023-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10676273/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138458121","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":"Generalized Extension of Referred Trigeminal Pain due to Greater Occipital Nerve Entrapment","authors":"Jung-woo Hyung, Byung-chul Son","doi":"10.1155/2023/1099222","DOIUrl":"https://doi.org/10.1155/2023/1099222","url":null,"abstract":"We report a very rare case of referred pain caused by greater occipital nerve (GON) entrapment, inducing spontaneous pain in the whole body as well as in the trigeminal nerve region of the face and head. It has already been reported that entrapment of the GON can induce referred pain in the ipsilateral limb as well as the ipsilateral hemiface. A 42-year-old female patient presented with chronic pain in her gums, jaw angle, submandibular region, retro-auricular suboccipital, and temporo-occipital vertex that had been ongoing for four years. As the patient’s head pain and facial pain became severe, severe spontaneous pain occurred in the arm, waist, and both lower extremities. This patient’s pain in the occipital and neck, spontaneous pain in the face, jaw, and whole body improved with decompression of the GON. Anatomical basis of pain referral to the facial trigeminal area caused by chronic GON entrapment is convergence of nociceptive inflow from high cervical C1–C3 structures and trigeminal orofacial area in the dorsal horn of the cervical spinal cord from the C2 segment up to the medullary dorsal horn (MDH). The major afferent contribution among the suboccipital and high cervical structure is mediated by spinal root C2 that is peripherally represented by the GON. Chronic noxious input from GON entrapment can cause sensitization and hypersensitivity in second order neurons in the trigeminocervical complex (TCC) and MDH in the caudal trigeminal nucleus and high cervical cord. Generalized extension of referred pain due to GON entrapment is thought to involve two possible pathophysiologies. One is the possibility that generalized pain is caused by sensitization of third-order nociceptive neurons in the thalamus. Another speculation is that spontaneous pain may occur throughout the body due to dysfunction of the descending brain stem pain-modulating pathway by sensitization and hyperexcitation of the MDH and trigeminal brainstem sensory nuclear complex (TBSNC).","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"41 12","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135041972","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Inadvertent Intrathecal Administration of Digoxin, with Review of the Literature.","authors":"Burton J Tabaac, Ian O T Laughrey, Hany F Ghali","doi":"10.1155/2023/4034919","DOIUrl":"https://doi.org/10.1155/2023/4034919","url":null,"abstract":"<p><p>While the systemic effects of digoxin have been studied, limited data exist on the effects of neuraxial administration. Prior case reports document how digoxin and lidocaine share indistinguishable vials and were inadvertently stocked together in spinal and epidural anesthesia kits, necessitating a need for further implementation of safety measures. Here, we report the poor progression and brain death of a postpartum woman after accidental administration of intrathecal digoxin during a routine elective cesarean section (C-section). It is imperative that quality improvement and safety measures are taken to avoid the recurrence of this medical error.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2023 ","pages":"4034919"},"PeriodicalIF":0.9,"publicationDate":"2023-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10611538/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71410689","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":"Alzheimer's Type Neuropathological Changes in a Patient with Depression and Anxiety: A Case Report and Literature Review of Neuropathological Correlates of Neuropsychiatric Symptoms in Alzheimer's Disease.","authors":"Sumit Das","doi":"10.1155/2023/5581288","DOIUrl":"https://doi.org/10.1155/2023/5581288","url":null,"abstract":"<p><p>Alzheimer's disease (AD) is classified as a tauopathy and is the most common neuropathological correlate of dementia/cognitive impairment. AD is neuropathologically characterized by the presence of beta-amyloid immunoreactive senile plaques and tau positive neurofibrillary tangles. Neuropsychiatric symptoms of AD however continue to be underscored, and therefore, neuropathological correlates of these neuropsychiatric symptoms are not readily studied. Presented here is a case of 60-year-old female who initially presented with anxiety and depression, and continued to be the predominant symptoms although mild cognitive impairment was noted as per the available clinical notes. Postmortem examination of the brain revealed severe Alzheimer's type neuropathological changes, which included significant tau and beta-amyloid pathology in limbic regions, which were thought to represent correlates of the patient's depression and anxiety. This case report illustrates the possible neuropathological correlates of neuropsychiatric symptoms in patients with AD. The author hopes that such a case will promote more in-depth studies into the pathophysiology of neuropsychiatric manifestations in AD.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2023 ","pages":"5581288"},"PeriodicalIF":0.9,"publicationDate":"2023-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10584483/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49674661","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}
Marta Rodrigues de Carvalho, Myrian Mathildes Sá de Deus Rocha, Vinícius Alves Bezerra, Maciel Eduardo de Pontes, Maria Cristina Del Negro, Julio Salgado Antunes, Vinícius Viana Abreu Montanaro, Rubens Nelson Morato Fernandez
{"title":"Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme A Reductase Immune-Mediated Necrotizing Myopathy following mRNA SARS-CoV-2 Vaccination.","authors":"Marta Rodrigues de Carvalho, Myrian Mathildes Sá de Deus Rocha, Vinícius Alves Bezerra, Maciel Eduardo de Pontes, Maria Cristina Del Negro, Julio Salgado Antunes, Vinícius Viana Abreu Montanaro, Rubens Nelson Morato Fernandez","doi":"10.1155/2023/7061783","DOIUrl":"https://doi.org/10.1155/2023/7061783","url":null,"abstract":"<p><p>The new coronavirus (COVID-19) pandemic has resulted in the unprecedented production of vaccines. In this context, the possible adverse effects remain to be identified and reported. In this article, we report the case of a young female patient who developed anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMG-CoA) immune-mediated necrotizing myositis (IMNM) after receiving the Pfizer-BioNTech (BNT162b2) COVID-19 vaccine. The diagnosis of probable post-vaccination IMNM was made due to the absence of other factors that may have led to the development of autoantibodies (medicines; e.g., statins, drugs) and the temporal relationship between exposure and event. This case report is the first to suggest that a COVID-19 vaccine may trigger anti-HMG-CoA reductase necrotizing myopathy.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2023 ","pages":"7061783"},"PeriodicalIF":0.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10239300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9585416","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}