Neurohospitalist最新文献

Background and Objectives: We report a rare case of severe posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) in an adult patient with hemophagocytic lymphohistiocytosis (HLH), and speculate that these three diagnoses are related by similar mechanisms of vascular endothelial dysfunction. Methods: Informed consent for this case report was obtained from the patient's legally authorized surrogate decision maker. Discussion and Practical Implications: Our patient initially presented with HLH secondary to intra-abdominal sepsis, and was later found to have severe PRES and RCVS resulting in extensive border-zone cortex infarction. Improvement of the severe systemic inflammatory syndrome characteristic of HLH and arrest of PRES and RCVS progression occurred only after HLH-specific treatment was initiated. In addition to illustrating the potential of HLH to manifest as PRES and RCVS, this case emphasizes the importance of prompt recognition and treatment of HLH and the role the neurologist can play in this process. This case also sheds light on the pathophysiological links between PRES, RCVS, and HLH. These three diagnoses may be related by similar mechanisms of vascular endothelial dysfunction caused by uncontrolled and severe systemic inflammation.

Background: syphilis is globally recognized as a great imitator due to its multiple manifestations and multi-organ involvement. This holds especially true in the context of neurosyphilis (NS), where stroke and other cerebrovascular manifestations are frequently overlooked. With the global reemergence of syphilis, meningovascular syphilis (MVS) and other vascular syphilitic affectations are now important, yet underdiagnosed causes of ischemic stroke. Purpose: this literature review focuses on syphilis in the context of stroke, examining the condition through this specific perspective. The pathophysiological aspect focuses on immune-mediated endothelial injury and vascular inflammation as main mechanisms leading to stroke. Analysis: a broader approach to syphilis is initially described, showcasing the comprehensive medical workup necessary for accurate diagnosis of MVS and special treatment considerations. Diagnostic challenges of NS are initially exposed, with cerebrospinal fluid (CSF) analysis and neuroimaging playing critical roles. While CSF-VDRL remains the gold standard, although, its low sensitivity necessitates a multimodal diagnostic approach combining serological, clinical, and radiographic findings. MRI and angiographic studies often reveal concentric steno-occlusive arteriopathy, most commonly affecting the middle cerebral and basilar arteries. Early recognition is vital, as NS can mimic common neurovascular etiologies, particularly in the context of younger adults without traditional risk factors. Treatment involves intravenous penicillin G, corticosteroids and antiplatelet agents playing supportive roles. However, clinicians must weigh bleeding risks in specific cases, particularly in patients with syphilitic aneurysmal disease. Conclusions: timely diagnosis and treatment of NS and MVS are essential to prevent irreversible neurological damage and contribute to the reduction of global stroke burden.

Introduction: Low levels of Vitamin D are associated with severe manifestations of autoimmune or inflammatory diseases; there is limited information regarding Guillain-Barré Syndrome (GBS).

Objective: To determine the serum levels of vitamin D in patients with GBS compared with healthy controls.

Materials and methods: A prospective observational study of consecutive patients with GBS based on EAN/PNS criteria over a year, from a single center was conducted. Clinical and paraclinical characteristics were obtained from the included patients upon admission; we determined the serum levels of Vitamin D (ng/ml) at admission and categorized them according to Vitamin D levels: sufficient >30 ng/mL, insufficient 20-30 ng/mL, and deficient <20 ng/mL. Poor prognosis was considered as non-independent walking at 3 months follow-up.

Results: The study included 56 patients with GBS (Guillain-Barré Syndrome) and 56 healthy control patients. The control group exhibited higher median levels of vitamin D compared to the GBS patient group [(29.9 ng/dl (IQR 24-34.8) vs 17.1 ng/dl (IQR 13.7-23.8 ng/dl), P < 0.001]. Only 9% (95% CI 1-19%) of the GBS patients had sufficient levels of vitamin D. In correlation analysis, significant differences were found between Vitamin D levels and glucose levels (r2 = -.36, P = 0.007) and the glucose-leukocyte index (GLI) (r2 = -.42, P=<0.001). In comparative analysis (Vitamin D levels ≤15 ngs/ml/ vs ≥ 16 ng/mL), the presence of dysautonomias, facial diparesis, leukocytes, glucose-leukocyte index (GLI), and glucose levels were significant; in the Kaplan-Meier survival analysis, patients with Vitamin D levels ≤15 ngs/ml showed lesser recovery in independent walking at 3 months (log-rank = 0.047).

Conclusion: Patients with GBS and low levels of Vitamin D exhibit a higher frequency of dysautonomias, higher GLI, and lesser recovery of independent walking.

Late-onset Pompe disease is a rare autosomal recessive lysosomal storage disorder caused by acid α-glucosidase deficiency, resulting in progressive skeletal muscle weakness and respiratory failure. We present the case of a 43-year-old African American woman who was admitted to the intensive care unit with acute-on-chronic hypoxemic and hypercarbic respiratory failure, alteration of consciousness, and progressive weakness. Her recent medical history included respiratory distress and aspiration pneumonia, which had not fully resolved despite supplemental oxygen therapy. On admission, initial evaluations including imaging and laboratory tests did not reveal a diagnosis. Muscle biopsy showed a vacuolar myopathy with excess glycogen suggestive of glycogen storage disease. Enzyme testing was obtained through the dried blood spot testing and was low. Molecular genetic testing identified two pathogenic variants in the GAA gene, confirming the diagnosis of late-onset Pompe disease. This diagnosis enabled the prompt initiation of enzyme replacement therapy (ERT) with alglucosidase alpha. The early initiation of ERT in this patient was pivotal in managing her condition, given the progressive nature of late-onset Pompe disease and the potential for improved outcome when treatment is started early. This case highlights the importance of considering late-onset Pompe disease in adults presenting with unexplained progressive respiratory and neuromuscular symptoms. It also demonstrates the critical role of biochemical and molecular genetic testing, as early intervention can significantly impact treatment outcomes and quality of life.

Background: Thyrotoxic periodic paralysis (TPP) is a rare, life-threatening, reversible condition, in which episodic muscle weakness occurs due to low serum potassium in the setting of thyrotoxicosis. In addition to treating thyrotoxicosis, prompt restoration of potassium levels is essential while monitoring for rebound hyperkalemia. Purpose: To emphasize the importance of recognizing and appropriately managing TPP, with a focus on the potential dangers of potassium overcorrection. Case Report: We present a patient with TPP whose potassium replacement therapy caused dangerous hyperkalemia, requiring aggressive potassium shifting and wasting therapies and vasopressor support. Conclusions: This case report highlights the importance of prompt recognition and treatment of TPP as well as the need for careful management of potassium levels to prevent respiratory failure and cardiac arrhythmias. We discuss the challenges associated with potassium repletion in thyrotoxicosis including the importance of careful monitoring and titration of potassium replacement therapy to avoid overcorrection and hyperkalemia.

This article highlights herpes zoster radiculitis, a condition that predominantly affects elderly patients and classically presents with progressive limb weakness, pain, and vesicular rash. Neuroimaging with MRI may demonstrate nerve root enlargement with T2-hyperintensity and gadolinium enhancement. Diagnosis can be confirmed with varicella zoster virus PCR in CSF. Effective treatment includes IV acyclovir and neuropathic pain agents such as gabapentin, which together can lead to symptomatic improvement. Early diagnosis and comprehensive treatment are crucial to prevent complications. Patients with zoster-associated plexopathy have a higher incidence of postherpetic neuralgia compared to those with herpes zoster rash alone. Thus, multimodal treatment involving antivirals, rehabilitation, and pain management is essential for recovery.

A 66-year-old female patient presented with progressive short-term memory loss over a period of three months and mild gait imbalance. MRI of the brain demonstrated symmetric expansile T2 FLAIR hyperintensities within the bilateral mesial temporal lobes, thalami, and cingulate gyri (Figure 1). Due to the symmetry of the signal changes in the bilateral cerebral hemispheres and especially the mesial temporal lobes, an autoimmune encephalitis was strongly favored on imaging. Glioblastoma was a consideration on the initial scan; however, it was thought to be much less likely, and the patient received immunosuppression with plasmapheresis and IV steroids. At that time, it was even presumed that the patient improved mildly with plasmapheresis. The patient was discharged on PO steroids; however, a few weeks later the patient presented in status epilepticus. On repeat MRI brain, findings were not significantly changed, and the diagnosis of an autoimmune process was again favored on imaging. The patient received plasmapheresis and IV steroids. However, on the second admission the patient's neurologic function was markedly below baseline per the family's report. The patient had received an extensive autoimmune, infectious and metabolic work up, including testing for Creutzfeldt-Jacob disease, with all the tests coming back negative. Therefore, a brain biopsy was performed to understand the underlying pathology, IDH wild-type glioblastoma. On MRI, the expansile signal changes were bilateral and multifocal, affecting more than three cerebral lobes. This is a case of gliomatosis cerebri, which was misdiagnosed as autoimmune encephalitis due to the symmetry of cerebral involvement.

A 13-year-old boy developed right-sided Horner's syndrome following resection of a benign mediastinal schwannoma extending from T1 to T3. Postoperatively, he exhibited ptosis, miosis, and anhidrosis, confirmed by starch iodine testing. The tumor likely involved the upper thoracic sympathetic ganglia-a rare site for schwannomas. This image highlights a rare iatrogenic cause of preganglionic Horner's syndrome. While Horner's is classically associated with apical lung or cervical lesions, this case emphasizes the importance of recognizing postoperative Horner's syndrome as a clinical clue to cervicothoracic sympathetic injury. It highlights the value of anatomical-clinical correlation in localizing lesions along the sympathetic pathway.

Movement disorders may be a core feature of autoimmune brain disorders, including paraneoplastic neurological syndromes. A 73-year-old white male presents with memory impairment, gait instability, dysphagia, and severe dysarthria progressing over 1.5 years. He recently developed behavior and sleep disturbances. His cognitive assessment showed time disorientation, short-term memory impairment with preserved retrieval, and pseudobulbar affect. The remainder of the neurological exam showed seborrhea, facio-cervical dystonia, a right positive palmomental reflex, fragmented pursuit eye movements, dysarthria, minor right pyramidal signs, bilateral asymmetric limb ataxia and symmetric akinetic parkinsonism. He was wheelchair-bound, capable of only a few short steps with help. MRI depicted generalized cortico-subcortical atrophy with temporal predominance. He was positive for antibodies anti-Hu in serum (1:10 000) and CSF (1:100), as antibodies anti-NMDAR in serum (1:320) and CSF (1:10). The patient had no clinical improvement after a therapeutical trial with 1 g intravenous methylprednisolone. Additional workup for occult neoplasia was positive for prostatic adenocarcinoma. He remains stable 2.5 years after disease onset.

Introduction: Early neurological deterioration (END) in ischemic stroke (IS) patients is a common complication that significantly impacts functional and vital prognoses. We aimed to determine the prevalence, predictors, and 90-day outcomes of END in Tunisian stroke patients.

Materials and methods: This was a retrospective cohort study of consecutive stroke cases admitted at the Neurology Department of the University Hospital in Monastir over 5 years, from 2018 to 2022. We included patients with an increment of two or more points on the NIHSS score within the first 7 days following IS. Univariate analysis and binary logistic regression were performed to identify independent factors associated with END.

Results: We included 489 patients with a mean age of 64 years (24 to 90) and a male predominance (sex ratio M/F = 1.86). The prevalence of END was 12.06% (59/489 patients). An elevated initial NIHSS score (OR = 1.13; 95% CI = 1.05-1.21), anterior choroidal artery stroke (OR = 5.39; 95% CI = 1.99-14.55), and large artery atherosclerosis (OR = 2.85; 95% CI = 1.17-6.9), were found to be independent factors associated with END. The causes of END included IS recurrence (12%), hemorrhagic transformation (10%), brain edema (10%), and stroke progression (68%). At 90 days, 80.8% (P < 0.001) of patients who experienced END had a mRS score of 2 or more, with a mortality rate of 18.6% (P < 0.001).

Conclusion: Patients with elevated NIHSS scores, AChA strokes, or LAA, should be prioritized for close observation during the acute phase. Enhancing our understanding of the predictive factors of END following IS could help target patients at very high risk of END and facilitate the development of more effective and rigorous strategies for prevention and treatment.