Neurohospitalist最新文献

Neuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory autoimmune disorder of the central nervous system, with optic neuritis and transverse myelitis as its most common presentations. Although immunomodulatory treatment options for NMOSD have expanded, preventing reactivation of latent infections in patients can be both a therapeutic challenge and a special consideration for the neurohospitalist in an inpatient setting. We present a challenging case of a NMOSD patient who presented to the emergency department with worsening weakness and numbness in the setting of an NMOSD pseudo-relapse, later found to have untreated latent tuberculosis (TB) and chronic hepatitis B (HBV). She was briefly treated with high-dose IV methylprednisolone, which was stopped after her symptoms and imaging became more consistent with a pseudo-relapse. After confirmation that neither HBV nor TB had reactivated, the patient was discharged on isoniazid and entecavir. A month later, the patient's symptoms were stable, and she was started on inebilizumab for relapse prevention of NMOSD. This case report is the first to highlight the therapeutic complexities of managing NMOSD that requires immunosuppression in the setting of preventing reactivation of both TB and HBV.

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.

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.

Background: Nitrous oxide (N₂O) has been an increasingly popular recreational drug over the past few years. Abuse is associated with severe neurological complications and even fatal outcomes. Purpose: Here we present a case of chronic nitric oxide abuse in a teenager presenting with rapidly progressive mixed sensory and motor polyneuropathy. Results: 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). Conclusion: The association of neurologic and genetic findings with the timeline of nitrous oxide inhalation suggests a multifactorial etiology of her symptoms, with the N₂O acting as a trigger to the axonal degeneration and demyelination detected on electrodiagnostic studies.

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.

Objectives: 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.

Methods: 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.

Results: 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 (P = .0002).

Conclusions: 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.

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.

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.

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.

Background and purpose: 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.

Methods: 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.

Results: 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.

Conclusion: 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.