Neurohospitalist最新文献

Background: The differential for acute onset progressive leukoencephalopathy in adults is broad. Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia is a rare genetic white matter disorder with typical onset around 40 years. Variability in clinical presentation can often lead to misdiagnosis with other neurodegenerative disorders, underscoring the importance of taking a detailed medical history, obtaining comprehensive diagnostic evaluations, and considering timely genetic testing.

Case presentation: A 53-year-old woman with a medical history of systemic lupus erythematosus and marginal zone B-cell lymphoma in remission presented with subacute onset fatigue, confusion, and slurred speech following SARS-CoV2 infection. Diagnostic testing was unremarkable except for elevated CSF interleukin-6, tumor necrosis factor, and myelin basic protein levels. The patient was diagnosed with presumed post-infectious encephalitis. Over the next 2 months, the patient's clinical syndrome progressed to include bradykinesia, hypophonia, dysphagia and resting tremor. Pathology and genetic testing revealed a rare diagnosis of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP).

Conclusions: This case illustrates a stepwise process for constructing a comprehensive differential diagnosis for acute onset of progressive leukoencephalopathy and a general management strategy. We also report a novel radiographic finding and genetic variant in the CSF1R gene associated with ALSP.

Background and purpose: Informed consent (IC) practices for endovascular thrombectomy (EVT) in acute stroke are not well elucidated. We investigated the roles and specialties of those obtaining EVT IC, aiming to provide insights for enhancing the process.

Methods: We conducted a survey from July- December 2023 among acute stroke care clinicians. Utilizing Qualtrics, we disseminated a questionnaire through various national and international online platforms. This analysis summarizes the characteristics of individuals who obtain IC at respondents' institution.

Results: Among 168 respondents, 71% were staff physicians, 70% practiced in the US and 70% worked at academic centers. Neurology (77%) was the most common specialty obtaining EVT IC, followed by neurosurgery (41%), radiology (30%) and emergency medicine (EM) (10%). Staff physicians were the most frequently involved (61%), followed by fellows (43%), residents (48%) and advanced practice providers (APPs) (36%). Comparatvely, non-US institutions were more likely to utilize neurologists alone (50% vs 31%, P = 0.016) and staff physicians (76% vs 54%, P = 0.008), while US institutions more often utilized neurosurgeons (51% vs 18%, P < 0.001), APPs (43% vs 18%, P = 0.002) and residents (56% vs 28%, P = 0.001). Non-academic institutions used EM (25% vs 5%, P < 0.001) and APPs (50% vs 31%, P = 0.031), while academic institutions commonly utilized neurosurgeons (48% vs 18%, P = 0.001), residents (59% vs 13%, P < 0.001) and fellows (52% vs 18%, P < 0.001).

Conclusion: Neurologists and staff physicians are the primary providers obtaining EVT IC, with variations based on region and institution type. Future efforts to optimize the IC process should integrate various specialties and be widely adaptable.

Baclofen, a GABA agonist, is used for the management of spasticity. Intrathecal route is indicated in cases of severe spasticity associated with spinal cord injury. Baclofen withdrawal symptoms can happen after IT pump removal which can present as anxiety, paranoia, psychosis, seizures, hallucinations, severe spasticity and dyskinesia. Cardiac arrest after baclofen withdrawal is rare with only 2 cases in literature so far however in both cases IT-pump was replaced. We present a case of PEA arrest after baclofen IT-pump removal which was managed without replacing. 33 year-old male with history of paraplegia from cervical spinal cord injury (C-6) level, chronic spasticity, fibular osteomyelitis and chronic sacral ulcers presented with wound dehiscence and exposed baclofen pump which was replaced 4 weeks ago due to the end of the pump's life. After 24-48 h of removal of the baclofen pump, cardiovascular instability in the form of supra ventricular tachycardia with hypotension and altered mentation was noted. It evolved to progressive clinical worsening with stiffness of body, loss of responsiveness, and up-rolling of eyes. Patient underwent PEA, and ROSC after 3 rounds of CPR. He was subsequently intubated, mechanically ventilated and sedated with midazolam intravenously. Baclofen and clonazepam were started through the feeding tube. Over a period of 10 days, midazolam was weaned, and baseline clinical stability was achieved without IT-baclofen pump. Though rare, cardiac arrest can happen after baclofen withdrawal. High index of clinical suspicion is advised for long term baclofen pump patients after pump explant to prevent withdrawal symptoms and complications.

Schistosoma and Angiostrongylus are both parasites which can cause central nervous system manifestations in humans. These parasites live in several overlapping geographic areas as well as share several clinical features. Diagnosis and treatment can be difficult given these infections are rare, have non-specific symptoms, and have definitive testing that takes days to weeks to result. We present a case of myelitis secondary to Angiostrongylus cantonensis infection with false-positive Schistosoma serologies complicating the diagnostic process in which the patient had an excellent clinical response to treatment with steroids and antiparasitic agents.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated optic neuritis is a demyelinating disease that shares clinical overlap with Neuromyelitis Optica Spectrum Disorder (NMOSD) and Multiple Sclerosis (MS). The immunosuppressive effects of pregnancy followed by a rebound in the immune system postpartum are thought to affect presentation and relapse rates of NMOSD and MS. Few studies exist describing pregnancy affecting MOG antibody-associated disease. In this case, a 29-year-old female from Ethiopia presented on postpartum day 8 with 4 weeks of progressive, painful bilateral vision loss that had acutely worsened over the past 6 days. Her visual acuity was light perception in the right eye and hand motion in the left eye with a right afferent pupillary defect. Dilated exam revealed bilateral optic nerve head elevation, vascular tortuosity, and macular folds. MRI of the brain and orbits with contrast revealed long segment bilateral optic nerve enhancement and nonspecific white matter changes. Serum MOG IgG was positive (1:160). Her vision rapidly improved with intravenous methylprednisolone 1000 mg daily for 5 days, followed by an oral prednisone taper. After 6 months, the patient's vision returned to 20/20 in each eye, and she has had no further clinical relapse. This case suggests that initial presentation of MOG optic neuritis may be affected by pregnancy. Physicians must appropriately triage patients with postpartum vision changes to ensure prompt treatment.

Background: Ataxia is a hallmark of the anti-GQ1b antibody syndrome, though it is unclear whether this symptom arises primarily from sensory nerve injury or from cerebellar involvement. We report a case of a patient with a clinically isolated post-infectious pancerebellar syndrome with anti-GQ1b antibody positivity.

Case presentation: A 22-year-old previously healthy woman presented with acute-onset, progressive imbalance, limb ataxia, and dysarthria following an upper respiratory tract infection. She had no paresthesias, numbness, or diplopia. Neurological examination revealed an isolated pancerebellar syndrome including ocular and limb dysmetria, intention tremor, and gait ataxia. All sensory modalities were unaffected, reflex testing was normal, and there was no ophthalmoplegia. In-hospital serum and CSF testing was unremarkable, and brain magnetic resonance imaging was normal. The patient was empirically treated with intravenous immunoglobulin. Her symptoms were moderately improved by discharge (hospital day 5), and fully resolved several months later. Serological testing sent during the hospitalization subsequently revealed anti-GQ1b IgG positivity.

Conclusions: This case expands the spectrum of anti-GQ1b syndrome to include isolated cerebellar ataxia, suggesting that ataxia in this syndrome can have cerebellar origin. Clinicians should consider anti-GQ1b testing in cases of acute cerebellar ataxia following infection.

Sickle cell disease (SCD) is a chronic hemoglobinopathy characterized by recurrent vaso-occlusive events and significant neurological morbidity. While ischemic and hemorrhagic strokes are well-known complications, reversible diffusion-restricting white matter lesions are exceedingly rare and underreported. We present the case of an 18-year-old male with homozygous SCD (HbSS) who developed acute neurological deterioration during a vaso-occlusive pain crisis. MRI revealed symmetric areas of restricted diffusion and FLAIR hyperintensities in the splenium of the corpus callosum and periventricular white matter, typically associated with cytotoxic edema and irreversible injury. Remarkably, the patient experienced near-complete neurological recovery with aggressive disease-targeted therapy, including hydroxyurea, voxelotor, and serial exchange transfusions. Follow-up MRI at 4 months showed complete resolution of the prior abnormalities. This case underscores the importance of recognizing potentially reversible diffusion-restricting lesions in SCD and challenges the conventional interpretation of restricted diffusion as a marker of permanent injury. Early recognition, comprehensive management, and serial neuroimaging may improve neurological outcomes in similar cases. Clinicians should maintain a high index of suspicion for reversible white matter injury when evaluating patients with sickle cell disease presenting with acute neurological symptoms. Incorporating serial neuroimaging and a multidisciplinary treatment approach is essential for timely diagnosis and optimizing neurological recovery in this vulnerable population.

Patients with acute psychiatric and cognitive symptoms can be challenging to evaluate, particularly in the context of renal or metabolic alterations. A 56-year-old woman initially presented with abdominal pain and vomiting, with no findings on physical examination. A severe chronic hypovolemic hypotonic hyponatremia was acknowledged in the lab work, leading to admission for sodium replacement and electrolyte imbalance correction. Two weeks later the patient developed altered mental status, agitation, psychosis and catatonia. This case highlights the diagnostic approach to patients with encephalopathy. Readers are guided through this stepwise approach, considering a wide range of differential diagnosis, including metabolic, infectious, autoimmune and paraneoplastic etiologies, with an extensive workup, ultimately arriving at the leading diagnosis.

Background/objective: The IRRAflow device combines intracerebroventricular (ICV) medication infusion, cerebrospinal fluid (CSF) irrigation, and continuous intracranial pressure (ICP) monitoring. While ICV milrinone is conventionally given as a bolus to manage vasospasm after aneurysmal subarachnoid hemorrhage (aSAH), this case presents the use of continuous ICV milrinone infusion via IRRAflow in a patient with refractory cerebral vasospasm following aSAH.

Results: A 47-year-old female with aSAH (Hunt Hess 2, Modified Fisher Grade 4) underwent coil embolization for a ruptured left PICA aneurysm. Despite standard management, severe vasospasm was detected in the bilateral middle cerebral arteries and basilar arteries on hospital day 6. Following initiation of systemic milrinone IV and intra-arterial verapamil treatment, subsequent transcranial Doppler (TCD) and CT angiogram revealed persistent vasospasm. Elevated ICPs precluded further angiography or ICV bolus therapy through the existing external ventricular drain (EVD). An IRRAflow catheter was inserted intraventricularly for continuous CSF drainage and ICV milrinone administration (2.6 mg/kg/day). Over days 8 to 12, vasospasm improved significantly, ICP normalized, and neurologic examination permitted extubation. Continuous ICV milrinone therapy was tapered over 5 days, and the IRRAflow system removed on day 14 without complications, leading to discharge for acute rehabilitation. Patient consent for case publication was documented per institutional protocol.

Conclusions: Continuous intrathecal milrinone infusion via IRRAflow may be a feasible adjunct for treating refractory vasospasm after aSAH. After the combined use of ICV milrinone via the IRRAflow catheter with standard-of-care therapies for severe vasospasm, the patient demonstrated favorable clinical and radiographic improvement without complications.

Background: We present a case report of a 24 year-old male who presented with encephalopathy, rigidity, and hyperthermia after receiving his medication. In this report, we guide you through the approach, differential diagnosis, investigation, and treatment modalities for similar presentations.