Case Reports in Neurological Medicine最新文献

{"title":"Amantadine as a Potential Treatment for Marchiafava–Bignami Disease: Case Reports and a Possible Mechanism","authors":"Leenil C Noel, Martin A. Myers, Tigran Kesayan","doi":"10.1155/2022/4585206","DOIUrl":"https://doi.org/10.1155/2022/4585206","url":null,"abstract":"Introduction Several reports have described the use of amantadine for managing symptoms in Marchiafava–Bignami disease (MBD); however, amantadine's role for the treatment of MBD symptoms is unclear. Here, we describe 2 patients with MBD who were treated with amantadine and hypothesize a potential mechanism responsible for clinical benefit. Case 1. A 38-year-old woman with excessive wine drinking presented with agitation, impaired speech, and a minimally conscious state. MRI revealed lesions in the splenium and genu. After being diagnosed with MBD, she was treated with intravenous thiamine, multivitamins, and 100 mg of amantadine twice a day for 2 weeks. She recovered to near baseline after 3 weeks. Case 2. A 54-year-old woman with years of heavy alcohol use presented with sudden bradyphrenia, acalculia, disinhibited behavior, weakness, and urinary incontinence. MRI revealed a large anterior callosal lesion. Two years after initial recovery from MBD, she noted that consuming “energy drinks” resulted in a transient, near-complete resolution of her residual behavioral, fatigue, and language symptoms. 100 mg of amantadine twice a day was trialled. After noted improvement, a further escalation to 200 mgs 3 times a day resulted in significant improvement in language and behavioral symptoms. Conclusion Amantadine in addition to vitamins may be beneficial in the treatment of MBD. It is possible that the dopaminergic effect of amantadine leads to improved recovery and function in dopamine-mediated pathways, including mesocortical and mesolimbic pathways during initial recovery, as well as improved speech, behavior, and fatigue in the following months. The role of amantadine in the treatment of MBD warrants further study.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"88 1","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79732714","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":"Artery of Percheron Infarction: A Case Report of Bilateral Thalamic Stroke Presenting with Acute Encephalopathy","authors":"C. Donohoe, Nooshin Kiani Nia, P. Carey, Vamsi Vemulapalli","doi":"10.1155/2022/8385841","DOIUrl":"https://doi.org/10.1155/2022/8385841","url":null,"abstract":"The artery of Percheron (AOP) is a relatively rare anatomic variant in which a solitary arterial trunk branches from the proximal segment of the posterior cerebral artery and provides arterial supply to the paramedian region of the thalami bilaterally and often to the rostral part of the midbrain. Occlusion of the artery of Percheron results in bilateral paramedian thalamic infarcts with and without midbrain involvement. Recognition of this condition as an acute stroke may be challenging due to various nonlocalized clinical presentations, given the wide range of neurological functions subserved by the thalamus. Prompt neuroimaging, preferably with magnetic resonance imaging (MRI), in conjunction with familiarity with this relatively rare vascular variation can facilitate initiation of appropriate time contingent thrombolytic treatment and improved long-term prognosis. We present a case of a 56-year-old African American female with a bilateral thalamic infarct secondary to the artery of Percheron thromboembolism. This patient presented unresponsive without focal neurologic findings but with an initial Glasgow Coma Score (GCS) of 7, and subsequent computed tomographic (CT) head revealed bilateral thalamic hypodensities. Confirmatory MRI exhibited bilateral subacute thalamic infarcts, which were thought to be embolic with the source from the left ventricular thrombus as the patient had at least three distinct clots. Unfortunately, the patient's mental status did not improve significantly, and she was discharged to a nursing facility for extended care. AOP infarction may be missed on vascular imaging utilizing CT, MRI, and even catheter angiography. Clinical recognition that the AOP is one of the only single artery occlusions that can affect bilateral structures and frequently present solely as altered mental status without focal neurologic deficits is crucial to the diagnosis.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"20 1","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84144154","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":"Fatigue and Exercise Intolerance as Initial Manifestations of a Nonsyndromic Mitochondrial Disorder Due to the Variant m.3243A>G","authors":"J. Finsterer, Sinda Zarrouk","doi":"10.1155/2022/7846852","DOIUrl":"https://doi.org/10.1155/2022/7846852","url":null,"abstract":"Objectives Fatigue and exercise intolerance have been only rarely reported as initial- and sole-onset manifestations of a mitochondrial disorder (MID). We present a patient with nonsyndromic MID with fatigue and exercise intolerance as its initial manifestations of the disease. Case Report. A 39 yo female experienced fatigue since age 18 and exercise intolerance since age 21. Later on, she developed Hashimoto thyroiditis, recurrent diffuse headache, and double vision upon exercise. Clinical exam revealed short stature, bilateral ptosis, partially reduced tendon reflexes, and hypertrophic calves. Serum lactate was elevated, and the lactate stress test was abnormal. Workup for suspected MID revealed ragged-red fibers and NADH-deficient muscle fibers, and biochemical investigations revealed a mild complex-I defect. mtDNA sequencing revealed the variant m.3243A>G with a heteroplasmy rate of 70% in the muscle. Conclusions This case shows that the initial manifestation of a MID can be fatigue and exercise intolerance. MIDs due to the m.3243A>G variant may have a slowly progressive course and only delayed multisystem involvement. The variant m.3243A>G may not only manifest as syndromic MID, particularly MELAS but also as nonsyndromic phenotype. MIDs should be considered as differentials of chronic fatigue even if no other phenotypic manifestation of a MID is present.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"74 1","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75889139","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":"Early and Delayed Rebound Intracranial Hypertension following Epidural Blood Patch in a Case of Spontaneous Intracranial Hypotension","authors":"E. Jafari, M. Karaminia, M. Togha","doi":"10.1155/2022/5637276","DOIUrl":"https://doi.org/10.1155/2022/5637276","url":null,"abstract":"Background Spontaneous intracranial hypotension (SIH) is a secondary headache that has been attributed to a cerebrospinal fluid (CSF) leak. It may resolve spontaneously or require conservative treatment. An epidural blood patch (EBP) with autologous blood is performed in cases exhibiting an inadequate response to conservative methods. Rebound intracranial hypertension (RIH) can develop following an EBP in up to 27% of patients. It is characterized by a change in the headache features and is often accompanied by nausea, blurred vision, and diplopia. Symptoms commonly begin within the first 36 hours, but could develop over days to weeks. It is important to differentiate this rebound phenomenon from unimproved SIH, as the treatment options differ. Case Presentation. Here, we present an interesting case of a patient with SIH who was treated with EBP and developed both immediate RIH after 24 hours and delayed RIH 3 weeks following EBP. Conclusions Following EBP for treatment of SIH, new onset of headache having a different pattern and location should always be monitored for the occurrence of RIH. A lumbar puncture should be done if the symptoms of elevated CSF pressure become intolerable or if the diagnosis is uncertain. Lack of early diagnosis and treatment and differentiation from SIH can cause complications and could affect the optic nerves.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"4 3","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72465044","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":"Demyelinating Neurological Adverse Events following the Use of Anti-TNF-α Agents: A Double-Edged Sword","authors":"Miral H Gharib, M. AlKahlout, Beatriz Garcia Canibano, Dirk Theophiel Deleu, Hani Malallah AlEssa, S. AlEmadi","doi":"10.1155/2022/3784938","DOIUrl":"https://doi.org/10.1155/2022/3784938","url":null,"abstract":"Background Tumor necrosis factor antagonists (anti-TNF-α) are an established therapeutic option for several autoimmune and inflammatory bowel diseases. Despite their clinical effectiveness, neurological adverse events have been reported, and literature data suggest a potential role of anti-TNF-α in the induction of demyelination. Case Presentation. In this series, we present three cases of demyelination after the use of anti-TNF-α agents. The first case involved a 21-year-old man with HLA-B27 negative peripheral spondylarthritis who had been taking adalimumab for 2 years. He developed headache, urinary incontinence, and bilateral lower extremity numbness that progressed to the middle of the trunk for 2 days. Magnetic resonance imaging (MRI) showed multiple hyperintense enhancement lesions in the left paramedian anterior pons consistent with multiple sclerosis (MS). The second case included a 17-year-old woman who was on 2 years of adalimumab treatment for juvenile idiopathic arthritis and chronic anterior uveitis and developed new-onset dizziness and tremors. The clinical examination showed signs of cerebellar dysfunction. MRI findings were consistent with multiple sclerosis. The third case was a 34-year-old male who was on 5 years of infliximab treatment for ankylosing spondylitis when he developed left hand numbness and weakness. Cerebrospinal fluid (CSF) analysis and MRI findings were consistent with demyelination. Discontinuation of tumor necrosis factor antagonists (anti-TNF-α) resulted in resolution of the symptoms with no recurrence in the first case, but there was evidence of recurrence in the other 2 cases, where one was managed with rituximab and the second one improved with pulse steroid therapy. Conclusion Despite the small number of patients, our series adds to the growing body of evidence supporting a causal link between anti-TNF-α agents and demyelination. Thus, we can conclude that on suspicion of any neurological side effects, early discontinuation of the TNF-α blockers and requesting urgent MRI scan to confirm the diagnosis is of utmost importance.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"1205 ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72494887","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":"Magnetic Resonance-Guided Diagnosis of Spontaneous Intracranial Hypotension in a Middle-Aged Woman","authors":"Jordan Hughes, Briana Chavez","doi":"10.1155/2022/4438923","DOIUrl":"https://doi.org/10.1155/2022/4438923","url":null,"abstract":"Spontaneous intracranial hypotension (SIH) is a rare condition caused by a cerebrospinal fluid (CSF) leak. It is diagnosed by clinical features that include an orthostatic headache combined with imaging findings demonstrating intracranial hypotension and a CSF leak. We present the case of a 45-year-old woman with an orthostatic headache who was found to have a sagging brain with a downward-displaced cerebellum and pachymeningeal enhancement with gadolinium contrast. This was initially misidentified as a Chiari I malformation, but the constellation of symptoms and MRI findings were later recognized as characteristic of SIH. Diagnosis of SIH and a CSF leak was confirmed with CT myelography. She was treated with a nontarget epidural blood patch, and her symptoms resolved. An orthostatic headache, a sagging brain, and pachymeningeal enhancement on MRI are highly specific for SIH, raising suspicion for this uncommon and often missed diagnosis.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"1 1","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90071501","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":"A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy","authors":"Ramón Vega, Benjamín González, Kiara Ortiz, Viviana Martínez, D. Carmona, Ivonne Vicente, Javier Chapa, Á. Chinea","doi":"10.1155/2022/4311382","DOIUrl":"https://doi.org/10.1155/2022/4311382","url":null,"abstract":"A link between intractable hiccups, as the initial symptom, and a possible neuromyelitis optica spectrum disorder (NMOSD) diagnosis is confusing but vital and may not be made by health care providers (HCPs) if they are not aware of the 2015 NMOSD criteria. Early diagnosis and adequate treatment are essential to prevent disease progression. We report the case of a 46-year-old Puerto Rican female who presented intractable hiccups when she was 31 (in 2004). Almost 15 years passed since the initial symptom, and after two severe relapses, she received a formal NMOSD diagnosis in March 2019. Treatment started with rituximab 1000 mg IV in April 2019. However, a lack of response to treatment led to a switch to eculizumab therapy in August 2019. The patient had cervical and brain magnetic resonance imaging (MRI) conducted in June 2020, which depicted a remarkable decrease in swelling and hyperintensity within the cervical spinal cord with no enhancing lesions when compared with the first MRI from February 2019. In addition, the patient suffered no new relapses, an improvement regarding disability, and a reduction of the cervical spinal cord lesion size. Nonetheless, this substantial decrease does not occur on all NMOSD patients, but more awareness of the disease is needed, especially in Puerto Rico. This case illustrates the efficacy of eculizumab therapy and the importance of differentiating the clinical, histopathological, and neuroimaging characteristics that separate demyelinating autoimmune inflammatory disorders, such as NMOSD and multiple sclerosis (MS).","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"12 1","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76949640","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":"Diabetic Striatopathy: Case Report and Possible New Actors.","authors":"Chiara Mozzini,&nbsp;Raffaele Ghirardi,&nbsp;Mauro Pagani","doi":"10.1155/2022/4176419","DOIUrl":"https://doi.org/10.1155/2022/4176419","url":null,"abstract":"<p><p>Diabetic striatopathy is a very rare neurological complication of diabetes. We report the case of an 86-year-old woman with poorly controlled type 2 diabetes admitted to the internal medicine ward for sudden onset of altered sensorium and severe bilateral choreiform and ballistic movements. The precise pathophysiology of this condition is not well understood. Our communication aims to remind clinicians to consider the possibility of diabetic striatopathy when poor-controlled diabetic patients have sudden-onset choreiform and ballistic movements. Moreover, this case suggests the possibility that oxidative and endoplasmic reticulum stress may be involved in this process.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2022 ","pages":"4176419"},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9792229/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10509861","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":"Ipsilateral Limb Extension of Referred Trigeminal Facial Pain due to Greater Occipital Nerve Entrapment: A Case Report.","authors":"Byung-Chul Son,&nbsp;Changik Lee","doi":"10.1155/2022/9381881","DOIUrl":"https://doi.org/10.1155/2022/9381881","url":null,"abstract":"<p><p>We report a very rare case of referred pain associated with entrapment of the greater occipital nerve (GON) occurring not only in the ipsilateral hemiface but also in the ipsilateral limb. There is an extensive convergence of cutaneous, tooth pulp, visceral, neck, and muscle afferents onto nociceptive and nonnociceptive neurons in the trigeminal nucleus caudalis (medullary dorsal horn). In addition, nociceptive input from trigeminal, meningeal afferents projects into trigeminal nucleus caudalis and dorsal horn of C1 and C2. Together, they form a functional unit, the trigeminocervical complex (TCC). The nociceptive inflow from suboccipital and high cervical structures is mediated with small-diameter afferent fibers in the upper cervical roots terminating in the dorsal horn of the cervical cord extending from the C2 segment up to the medullary dorsal horn. The major afferent contribution is mediated by the spinal root C2 that is peripherally represented by the greater occipital nerve (GON). Convergence of afferent signals from the trigeminal nerve and the GON onto the TCC is regarded as an anatomical basis of pain referral in craniofacial pain and primary headache syndrome. Ipsilateral limb pain occurs long before the onset of the referred facial pain. The subsequent severe hemifacial pain suggested GON entrapment. The occipital nerve block provided temporary relief from facial and extremity pain. Imaging studies found a benign osteoma in the ipsilateral suboccipital bone, but no direct contact with GON was identified. During GON decompression, severe entrapment of the GON was observed by the tendinous aponeurotic edge of the trapezius muscle, but the osteoma had no contact with the nerve. Following GON decompression, the referred trigeminal and extremity pain completely disappeared. The pain referral from GON entrapment seems to be attributed to the sensitization and hypersensitivity of the trigeminocervical complex (TCC). The clinical manifestations of TCC hypersensitivity induced by chronic entrapment of GONs are diverse when considering the occurrence of extremity pain as well as facial pain.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2022 ","pages":"9381881"},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9734007/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10681860","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":"Optic Neuritis Presented as Syndrome of Inappropriate Antidiuretic Hormone Secretion in an 8 Year Old.","authors":"T G M Prasadani,&nbsp;Kapila Panditha,&nbsp;D Irugalbandara","doi":"10.1155/2021/6672827","DOIUrl":"https://doi.org/10.1155/2021/6672827","url":null,"abstract":"<p><p>Optic neuritis is a rare demyelinating disorder, which involves the optic nerve. It can be a monophasic self-limiting illness due to postinfectious or postvaccination etiology. It can also be an initial presentation of a relapsing demyelinating disorder such as multiple sclerosis or neuromyelitis optica spectrum of disorders. It is characterized to aquaporin-4 antibody-rich areas in the brain, optic nerve, and spinal cord. The hypothalamus and periventricular area are also rich in specific antibodies and may lead to dysfunction in the hypothalamic-pituitary axis. Antidiuretic hormone (ADH) is synthesized in the hypothalamus and stored in the posterior pituitary and may secrete inappropriately due to this disturbance. This will impair water excretion from the kidney, leading to hyponatremia. When hyponatremia is significant, the patient will present with confusion, agitation, and convulsions. This case report discusses acute symptomatic hyponatremia as the initial presentation of optic neuritis due to syndrome of inappropriate ADH secretion (SIADH).</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2021 ","pages":"6672827"},"PeriodicalIF":0.9,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435311/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10051597","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}