Huntington's disease: genetically programmed cell death in the human central nervous system

ABSTRACT This case is the first document to describe a patient receiving anti-programmed cell death 1 (PD-1) antibodies which showed cranial dura matter involvement. According to the increasing use of anti-PD-1 monoclonal antibodies, adverse effects can occur in several organs since its ligand PD-L1 and PD-L2 are expressed in a wide variety of tissues. The estimated rate of neurological complications is 1–4.2% of patients, and neuromuscular disorders are the most common. Adverse effects on the central nervous system including encephalitis are less frequent. Here, a patient receiving anti-PD-1 antibodies showed cranial dura matter involvement, and the dura enhancement on MRI was resolved by withdrawal of the treatment with anti-PD-1 antibodies only.

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Efficacy of PD-1 or PD-L1 inhibitors and central nervous system metastases in advanced cancer: a meta-analysis

Current Cancer Drug Targets ◽

10.2174/1568009621666210601111811 ◽

2021 ◽

Vol 21 ◽

Author(s):

Minyong Peng ◽

Shan Li ◽

Hui Xiang ◽

Wen Huang ◽

Weiling Mao ◽

...

Keyword(s):

Central Nervous System ◽

Overall Survival ◽

Cell Death ◽

Nervous System ◽

Programmed Cell Death ◽

Future Research ◽

Significant Heterogeneity ◽

Cns Metastases ◽

Hazard Ratios ◽

Significant Difference

Background: Little is known about the efficacy of programmed cell death protein-1 (PD-1) or programmed cell death-ligand 1 (PD-L1) inhibitors in patients with central nervous system (CNS) metastases. Objective: Assess the difference in efficacy of PD-1 or PD-L1 inhibitors in patients with and without CNS metastases. Methods: From inception to March 2020, PubMed and Embase were searched for randomized controlled trials (RCTs) about PD-1 or PD-L1 inhibitors. Only trails with available hazard ratios (HRs) for overall survival (OS) of patients with and without CNS metastases simultaneously would be included. Overall survival hazard ratios and their 95% confidence interval (CI) were calculated, and the efficacy difference between these two groups was assessed in the meantime. Results: 4988 patients (559 patients with CNS metastases and 4429 patients without CNS metastases) from 8 RCTs were included. In patients with CNS metastases, the pooled HR was 0.76 (95%CI, 0.62 to 0.93), while in patients without CNS metastases, the pooled HR was 0.74 (95%CI, 0.68 to 0.79). There was no significant difference in efficacy between these two groups (Χ=0.06 P=0.80). Conclusion: With no significant heterogeneity observed between patients with or without CNS metastases, patients with CNS metastases should not be excluded from PD-1 or PD-L1 blockade therapy. Future research should permit more patients with CNS metastases to engage in PD-1 or PD-L1 blockade therapy and explore the safety of PD-1 or PD-L1 inhibitors in patients with CNS metastases.

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Central nervous system-specific alterations in the tryptophan metabolism in the 3-nitropropionic acid model of Huntington's disease

Pharmacology Biochemistry and Behavior ◽

10.1016/j.pbb.2015.03.002 ◽

2015 ◽

Vol 132 ◽

pp. 115-124 ◽

Cited By ~ 9

Author(s):

Gábor Veres ◽

Máté Molnár ◽

Dénes Zádori ◽

Márton Szentirmai ◽

Levente Szalárdy ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Huntington's Disease ◽

Huntington’S Disease ◽

Tryptophan Metabolism ◽

Nitropropionic Acid ◽

3 Nitropropionic Acid

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Huntington's Disease: Two-Year Observational Follow-Up of Executive Function Evaluation with CNS Vital Signs Test in an Adult Patient

Case Reports in Medicine ◽

10.1155/2011/385894 ◽

2011 ◽

Vol 2011 ◽

pp. 1-4

Author(s):

Anna Lucia Spear King ◽

Alexandre Martins Valença ◽

Adriana Cardoso de Oliveira e Silva ◽

Ana Claudia Cerqueira ◽

Lígia Maria Chaves Ferraz ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Huntington's Disease ◽

Huntington’S Disease ◽

Vital Signs ◽

Clinical Severity ◽

Psychomotor Development ◽

Cognitive State ◽

Evolutionary Stage ◽

Medication Therapy

Huntington's disease (HD) is a genetic, degenerative, and progressive central nervous system disease. It is characterized by motor abnormalities and cognitive and psychiatric symptoms.Objective. To describe the precise degree of clinical severity of patients with HD through a new neurocognitive assessment.Methods. Unprecedented battery of computerized tests, CNSVS (Central Nervous System Vital Signs), was applied at three different moments in 2008, 2009, and 2010. The accurate and reliable CNSVS objectively provided the cognitive state of patients and allowed for the evaluation of disease progression.Case Report. P., 26, female, without any medication, with normal psychomotor development is a parent carrier of HD. In 2008, she was diagnosed with HD in accordance with the Medical Genetics Laboratories.Conclusion. The tests may be useful to reveal the exact measure of the current evolutionary stage of HD patients, allowing for more efficient planning of treatment and future procedures, such as the medication, therapy, and physical activity to be administered.

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