NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-06-08DOI: 10.1177/19418744231182285
Andrew Silverman, Rachelle Dugue, Paul M George
{"title":"Clinical Problem Solving: A 38-year-Old Woman With Systemic Lupus Erythematosus Presenting With Headache, Nausea, and Vomiting.","authors":"Andrew Silverman, Rachelle Dugue, Paul M George","doi":"10.1177/19418744231182285","DOIUrl":"10.1177/19418744231182285","url":null,"abstract":"<p><p>A 38-year-old woman with migraine headaches and systemic lupus erythematosus with recent cessation of her immunosuppressive therapy presents with prolonged headache and hypertensive emergency. Her examination is notable for a peripheral right facial palsy and stable malar rash. There are no signs of systemic infection nor systemic symptoms of a lupus flare. Initial CT head reveals bilateral hypodensities in the basal ganglia. Within 8 hours of presentation, she develops right hemiplegia and becomes encephalopathic. MRI shows multifocal acute infarcts (most notably in the left basal ganglia), enhancement of the right facial nerve, and multifocal vessel wall enhancement in the anterior and posterior circulation. We discuss the differential diagnosis, comprehensive workup, and subsequent treatment decisions in the management of this immunocompromised patient with encephalopathy, headache, and rapidly progressing focal neurologic deficits.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"394-398"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494810/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10295031","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-05-10DOI: 10.1177/19418744231175564
Valeria Ariza Hutchinson, Aya Shnawa, David P Lerner, Kenneth Wener, Anna Cervantes-Arslanian, Joseph D Burns
{"title":"Gummatous Neurosyphilis With Transient Worsening of Neurological Symptoms After Treatment Initiation.","authors":"Valeria Ariza Hutchinson, Aya Shnawa, David P Lerner, Kenneth Wener, Anna Cervantes-Arslanian, Joseph D Burns","doi":"10.1177/19418744231175564","DOIUrl":"10.1177/19418744231175564","url":null,"abstract":"<p><p>Cerebral syphilitic gumma is an atypical presentation of neurosyphilis, the clinical manifestations of which depend on the size and location of the lesions. It radiologically presents as enhancing nodular lesion(s) in brain parenchyma. We present a case of a patient with cerebral syphilitic gummas who had worsening neurological symptoms a few hours after initiation of anti-syphilitic antibiotic treatment. We aim to illustrate the clinical and radiological characteristics that might be helpful to clinicians when approaching the challenges they might encounter while treating neurosyphilis.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"403-405"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494825/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10232154","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-05-12DOI: 10.1177/19418744231174396
Giovanni Castellucci, Michelle Figueroa, Lalitha Sivaswamy
{"title":"Hereditary Neuropathy With Liability to Pressure Palsy Detected During the Use of Recreational Drugs.","authors":"Giovanni Castellucci, Michelle Figueroa, Lalitha Sivaswamy","doi":"10.1177/19418744231174396","DOIUrl":"10.1177/19418744231174396","url":null,"abstract":"<p><p><b>Background:</b> Nitrous oxide (N<sub>2</sub>O) has been an increasingly popular recreational drug over the past few years. Abuse is associated with severe neurological complications and even fatal outcomes. <b>Purpose:</b> Here we present a case of chronic nitric oxide abuse in a teenager presenting with rapidly progressive mixed sensory and motor polyneuropathy. <b>Results:</b> The initial diagnostic workup excluded electrolyte derangement, heavy metal intoxication, autoimmune neuropathy, myopathy, hematological disorders, and thyroid disease. On further questioning, patient reported 8-months of inhalation of nitrous oxide, commonly known as \"whippets\". Subsequent tests revealed low Vitamin B12 and elevated homocysteine level. Eventual genetic test demonstrated a heterozygous deletion in the gene that encodes the peripheral myelin protein 22 (PMP22), consistent with a diagnosis of Hereditary Neuropathy with Liability to Pressure Palsies (HNPP). <b>Conclusion:</b> The association of neurologic and genetic findings with the timeline of nitrous oxide inhalation suggests a multifactorial etiology of her symptoms, with the N<sub>2</sub>O acting as a trigger to the axonal degeneration and demyelination detected on electrodiagnostic studies.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"376-380"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494818/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10232155","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-07-10DOI: 10.1177/19418744231189078
Kristy M Phillips, Josanna M Rodriguez-Lopez, Andrew J Webb
{"title":"Elevations in Norclobazam Concentrations and Altered Mental Status in CYP2C19 Poor Metabolizer Phenotype: A Case Report.","authors":"Kristy M Phillips, Josanna M Rodriguez-Lopez, Andrew J Webb","doi":"10.1177/19418744231189078","DOIUrl":"10.1177/19418744231189078","url":null,"abstract":"<p><p>Clobazam is a 1,5-benzodiazepine frequently used as an adjunctive agent for refractory seizures and status epilepticus. Clobazam undergoes metabolism to an active metabolite norclobazam which is subsequently hydroxylated by CYP2C19, a cytochrome with several pharmacogenetic variants. Patients with poor metabolizer phenotypes may have elevated norclobazam levels and subsequent adverse effects. We present a case of an Asian American male receiving clobazam at a standard therapeutic dose for seizure disorder who became comatose secondary to significantly elevated norclobazam concentrations. Genetic testing revealed the patient was a poor CYP2C19 metabolizer, accounting for the impaired clearance. Clinicians should be aware of the patient populations at risk for these genetic polymorphisms and adjust initial doses based on package labeling or consider therapeutic drug monitoring to avoid adverse effects.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"434-437"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10239189","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-05-10DOI: 10.1177/19418744231173829
Katherine M Clifford, Connie K Wu, David Post, Ruba Shaik, Srikanth Muppidi
{"title":"Utility of Repetitive Nerve Stimulation in the Diagnosis of Myasthenia Gravis in the Inpatient Setting.","authors":"Katherine M Clifford, Connie K Wu, David Post, Ruba Shaik, Srikanth Muppidi","doi":"10.1177/19418744231173829","DOIUrl":"10.1177/19418744231173829","url":null,"abstract":"<p><strong>Objectives: </strong>Sensitivity and specificity of Repetitive Nerve Stimulation (RNS) is typically reported from outpatient centers, and we hypothesized that these values might not apply to hospitalized patients with higher grades of weakness. RNS may be helpful in rapidly confirming diagnosis of myasthenia gravis (MG) in the inpatient setting, as results from confirmatory antibody testing are often delayed. We sought to characterize the sensitivity and specificity of RNS in the inpatient setting to assist in the early diagnosis of MG.</p><p><strong>Methods: </strong>We performed a retrospective analysis of all adult patients who had inpatient RNS at our center from 2016 to 2021. Inclusion criteria included RNS performed at least at one site and a neurological evaluation which prompted an electrodiagnostic study to evaluate for neuromuscular junction (NMJ) pathology. Descriptive statistics and Fisher exact analysis were performed.</p><p><strong>Results: </strong>Of the 32 identified hospitalized patients, 6 had greater than 10% decrement on slow RNS, confirming NMJ dysfunction. Five were diagnosed with MG, and 1 with Lambert-Eaton myasthenic syndrome. Of the 26 patients with normal RNS, 25 ultimately had alternative causes of weakness. One was later diagnosed as seronegative MG based on clinical improvement with acetylcholinesterase inhibitors. In our inpatient population, the overall sensitivity and specificity of RNS were 83.3% and 96.2% respectively. There was a statistically significant association between a positive RNS and diagnosis of MG (<i>P</i> = .0002).</p><p><strong>Conclusions: </strong>RNS is a highly sensitive and specific test for the diagnosis of MG in an inpatient setting, and these results are likely more rapidly available compared to antibody testing.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"364-370"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494823/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10244489","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-05-29DOI: 10.1177/19418744231180046
Jakob V E Gerstl, Joshua D Bernstock, Ari D Kappel, Erickson F Torio, Rose Du
{"title":"Recurrent Intraventricular Haemorrhage in Cerebral Proliferative Angiopathy - A Case Report and Review of the Literature.","authors":"Jakob V E Gerstl, Joshua D Bernstock, Ari D Kappel, Erickson F Torio, Rose Du","doi":"10.1177/19418744231180046","DOIUrl":"10.1177/19418744231180046","url":null,"abstract":"<p><p>Cerebral proliferative angiopathy (CPA) is an entity distinct from that of classical arteriovenous malformations. As such, few reports have considered the long-term follow-up of patients with hemorrhage in CPA. Accordingly, herein the authors present a case of recurrent hemorrhage in CPA with 32 years of follow-up and in so doing summarize the literature of hemorrhagic cases in CPA. A 19-year-old presented with focal awareness seizures and diagnostic work-up revealed a left hemispheric vascular lesion. The patient presented again with intracranial hemorrhage at ages 28, 43 and 51. Angioarchitectural workup revealed intermingled brain parenchyma between vascular spaces, absence of dominant feeders and a clear nidus consistent with CPA. The size and diffuse nature of the lesion deemed it inoperable. Given our case and review of the literature it is apparent that CPA has a high risk of re-hemorrhage in the rare event that hemorrhage does occur.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"345-350"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494813/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10232151","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-05-11DOI: 10.1177/19418744231175561
Haley K Talbot-Stetsko, Sara Saleh, Ashley Brent, Sandra Camelo-Piragua, David Gordon, Craig A Williamson
{"title":"Vertebral and Basilar Artery Dissection in a Patient With Alport Syndrome.","authors":"Haley K Talbot-Stetsko, Sara Saleh, Ashley Brent, Sandra Camelo-Piragua, David Gordon, Craig A Williamson","doi":"10.1177/19418744231175561","DOIUrl":"10.1177/19418744231175561","url":null,"abstract":"<p><p>Basilar artery occlusion (BAO) is a rare cause of stroke associated with significant morbidity and mortality. It is most frequently thromboembolic in nature, but may be caused by vertebral artery dissection. We present a case of BAO in a 36-year-old woman with Alport syndrome. She was treated with emergent thrombectomy via the right vertebral artery with return to baseline neurological status. Her clinical status deteriorated later the same day and she was found to have re-occlusion. Repeat thrombectomy was complicated by persistent re-occlusion requiring 7 passes to achieve reperfusion. Unfortunately, her neurological exam remained poor and she was transitioned to comfort care, expiring on admission day 3. An autopsy demonstrated acute dissection of the left vertebral artery, basilar artery, and bilateral posterior cerebral arteries. Alport syndrome is a type IV collagenopathy most known for causing kidney disease. It may also be associated with vascular fragility as type IV collagen forms a significant component of the vascular basement membrane. There are reports of aortic, coronary, and cervical dissections, but few reports of intracranial dissections in patients with Alport syndrome. While iatrogenic dissection cannot be ruled out, the histological findings in this case are most consistent with spontaneous arterial dissection as the cause of her initial neurologic presentation. This highlights the need for further investigation into the relationship between Alport syndrome and vascular fragility and should alert clinicians to the possibility of intracranial dissection in patients with AS.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"410-414"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494822/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10232156","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-05-10DOI: 10.1177/19418744231174949
Karlos Acurio, Miguel Chuquilin
{"title":"Neuro-Sweet Syndrome: A Diagnostic Conundrum.","authors":"Karlos Acurio, Miguel Chuquilin","doi":"10.1177/19418744231174949","DOIUrl":"10.1177/19418744231174949","url":null,"abstract":"<p><p>Sweet Syndrome presents as acute fever, leucocytosis and characteristic skin plaques. It can involve many organ systems but rarely affects the nervous system. We report the case of a 51-year-old female that presented with fever, rash, headache and encephalopathy. Brain magnetic resonance imaging showed extensive T2 hyperintensities involving cerebral hemispheres, cerebellum, and brainstem. A skin biopsy revealed dermal infiltration by neutrophils consistent with Sweet Syndrome. She started steroid treatment with a good clinical response. Further questioning revealed that she had a similar episode 10 years prior that had been diagnosed as acute disseminated encephalomyelitis. Neuro-Sweet Syndrome can present with a great array of symptoms and relapses over long periods of time making the diagnosis difficult without a high degree of suspicion. Clinicians should consider this syndrome in the setting of acute encephalitis with white matter lesions that are highly responsive to steroids particularly in the presence of previous similar symptoms.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"406-409"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494816/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10242454","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-05-10DOI: 10.1177/19418744231174577
Shefali Dujari, Janet Wei, Lironn Kraler, Tarini Goyal, Eric Bernier, Neil Schwartz, Karen Hirsch, Carl A Gold
{"title":"Inpatient Neurology Deaths and Factors Associated With Discharge to Hospice.","authors":"Shefali Dujari, Janet Wei, Lironn Kraler, Tarini Goyal, Eric Bernier, Neil Schwartz, Karen Hirsch, Carl A Gold","doi":"10.1177/19418744231174577","DOIUrl":"10.1177/19418744231174577","url":null,"abstract":"<p><strong>Background and purpose: </strong>The Neurology Mortality Review Committee at our institution identified variability in location of death for patients on our inpatient neurology services. Hospice may increase the number of patients dying in their preferred locations. This study aimed to characterize patients who die on inpatient neurology services and explore barriers to discharge to hospice.</p><p><strong>Methods: </strong>This retrospective study was completed at a single, quaternary care medical center that is a Level I Trauma Center and Comprehensive Stroke Center. Patients discharged by an inpatient neurology service between 6/2019-1/2021 were identified and electronic medical record review was performed on patients who died in the hospital and who were discharged to hospice.</p><p><strong>Results: </strong>69 inpatient deaths and 74 discharges to hospice occurred during the study period. Of the 69 deaths, 54 occurred following withdrawal of life sustaining treatment (WLST), of which 14 had a referral to hospice placed. There were 88 \"hospice-referred\" patients and 40 \"hospice-eligible\" patients. Hospice-referred patients were less likely to require the intensive care unit than hospice-eligible patients. Hospice-referred patients had their code status changed to Do Not Intubate earlier and were more likely to have advanced directives available.</p><p><strong>Conclusion: </strong>Our data highlight opportunities for further research to improve discharge to hospice including interhospital transfers, advanced directives, earlier goals of care discussions, palliative care consultations, and increased hospice bed availability. Importantly, it highlights the limitations of using in-hospital mortality as a quality indicator in this patient population.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"337-344"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494814/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10244490","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}
NeurohospitalistPub Date : 2023-10-01Epub Date: 2023-07-25DOI: 10.1177/19418744231192159
Daniel J Zhou, Andria M Powers, Caleb A Cave, Emily K Dickas, Mary C Rickard, Geetanjali Rathore, Rhonda R Wright, Rana K Zabad, Sookyong Koh
{"title":"Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure.","authors":"Daniel J Zhou, Andria M Powers, Caleb A Cave, Emily K Dickas, Mary C Rickard, Geetanjali Rathore, Rhonda R Wright, Rana K Zabad, Sookyong Koh","doi":"10.1177/19418744231192159","DOIUrl":"10.1177/19418744231192159","url":null,"abstract":"<p><p>We report two distinct challenging initial presentations of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Case 1 describes a 12-year-old boy who developed headaches refractory to pain medication followed by cranial neuropathies and intracranial hypertension, confirmed by lumbar puncture with an opening pressure >36 cm H<sub>2</sub>O. Case 2 describes a 3-year-old boy who developed new-onset seizures refractory to antiseizure medications, a presentation of FLAIR-hyperintense lesions in MOG-antibody associated encephalitis with seizures (FLAMES). On repeat magnetic resonance imaging, both patients were found to have cortical T2 hyperintensities, leptomeningeal contrast enhancement, and bilateral optic nerve enhancement. In the cerebrospinal fluid, both patients had CSF pleocytosis with neutrophilic predominance. The patients were treated with intravenous immunoglobulins, plasma exchange, and high-dose corticosteroids. The first patient achieved disease remission, whereas the second patient required the addition of rituximab for management of seizures. The two cases highlight the pleomorphic clinical phenotypes of MOGAD.</p>","PeriodicalId":46355,"journal":{"name":"Neurohospitalist","volume":"13 4","pages":"438-444"},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10239194","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}