Paraneoplastic Neurological Syndromes as Initial Presentation of Tumors: An Eight-Year Single-Center Experience.

BACKGROUND: Paraneoplastic Neurological Syndromes (PNS) comprise a diverse group of disorders propagated by immune-mediated effects of malignant tumors on neural tissue. METHODS: A single-center longitudinal study was performed including consecutive adult patients treated at a tertiary academic hospital between 2015 and 2023 and diagnosed with PNS. PNS were ascertained using the 2004 and the revised 2021 PNS-Care diagnostic criteria. RESULTS: Thirteen patients who fulfilled the 2004 definite PNS criteria were included. PNS comprise diverse neurological syndromes, with neuromuscular junction disorders (54%) and limbic encephalitis (31%) being predominant. PNS-related antibodies were detected in 85% of cases, including anti-AChR (n = 4), anti-P/Q-VGCC (n = 3), anti-Hu (n = 3), anti-Yo (n = 1), anti-Ma (n = 1), anti-titin (n = 1), anti-IgLON5 (n = 1), and anti-GAD65 (n = 1). Thymoma (31%), small-cell lung cancer (23%), and papillary thyroid carcinoma (18%) were the most frequent tumors. Imaging abnormalities were evident in 33% of cases. Early immunotherapy within 4-weeks from symptom onset was associated with favorable outcomes. At a mean follow-up of 2 ± 1 years, two patients with anti-Hu and anti-Yo antibodies died (18%). Four and three patients fulfilled the 2021 PNS-Care diagnostic criteria for definite and probable PNS, respectively. CONCLUSIONS: This study highlights the clinical heterogeneity of PNS, emphasizing the need for early suspicion and prompt treatment initiation for optimal outcomes.

A Brief Review of Brain's Blood Flow-Metabolism Coupling and Pressure Autoregulation.

BACKGROUND: The human brain, depending on aerobic glycolysis to cover its metabolic needs and having no energy reserves whatsoever, relies on a constant and closely regulated blood supply to maintain its structural and functional integrity. Cerebral autoregulation, that is, the brain's intrinsic ability to regulate its own blood flow independently from the systemic blood pressure and cardiac output, is an important physiological mechanism that offers protection from hypoperfusion injury. DISCUSSION: Two major independent mechanisms are known to be involved in cerebral autoregulation: (1) flow-metabolism coupling and (2) myogenic responses of cerebral blood vessels to changes in transmural/arterial pressure. A third, less prominent component of cerebral autoregulation comes in the form of neurogenic influences on cerebral vasculature. CONCLUSION: Although fragmentation of cerebral autoregulation in separate and distinct from each other mechanisms is somewhat arbitrary, such a scheme is useful for reasons of simplification and to better understand their overall effect. Comprehension of cerebral autoregulation is imperative for clinicians in order for them to mitigate consequences of its impairment in the context of traumatic brain injury, subarachnoid hemorrhage, stroke, or other pathological conditions.