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Head and voice tremor improving with immunotherapy in an anti-NF155 positive CIDP patient.

ABSTRACT: Chronic inflammatory demyelinating polyradiculoneuropathy with NF155 antibodies (anti-NF155+) constitutes a specific chronic inflammatory demyelinating polyradiculoneuropathy subset with a high incidence of limb's tremor and poor response to conventional therapies. We report a patient with chronic inflammatory demyelinating polyradiculoneuropathy anti-NF155+ with a severe tremor involving limbs, head and voice that responded very well to rituximab. This response correlated with a sharp decrease in the anti-NF155 titers. This case is the first report associating head and voice tremor to chronic inflammatory demyelinating polyradiculoneuropathy, reinforces the hypothesis of the cerebellar origin of this tremor and provides indirect evidence that the antibodies may be the cause of the tremor in these patients.

SUBMITTER: Painous C

PROVIDER: S-EPMC5899910 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature

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Head and voice tremor improving with immunotherapy in an anti-NF155 positive CIDP patient.

Painous Cèlia C López-Pérez Mª Ángeles MÁ Illa Isabel I Querol Luis L

Annals of clinical and translational neurology 20180307 4

Chronic inflammatory demyelinating polyradiculoneuropathy with NF155 antibodies (anti-NF155+) constitutes a specific chronic inflammatory demyelinating polyradiculoneuropathy subset with a high incidence of limb's tremor and poor response to conventional therapies. We report a patient with chronic inflammatory demyelinating polyradiculoneuropathy anti-NF155+ with a severe tremor involving limbs, head and voice that responded very well to rituximab. This response correlated with a sharp decrease ...[more]

PMID: 29687027

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Project description:ImportanceEssential vocal tremor (EVT) is a neurologic voice disorder characterized by periodic fluctuations in pitch and loudness that can hinder intelligibility. Defining the normative range of vocal tremor frequency may assist in diagnosis and provide insight into disease mechanisms.ObjectiveTo characterize the normative voice tremor frequency in EVT (in hertz).Design, setting, and participantsCross-sectional observational study of 160 patients with EVT. The setting was a tertiary voice center. Participants were identified from a database of consecutive patients diagnosed as having laryngeal movement disorders between January 1, 1990, and April 1, 2017.Main outcomes and measuresThe following 3 methods measured the frequency of tremor experienced by patients with EVT: perceptual method, computerized peak detection method, and laryngeal electromyography method. Within-person and population-level tremor frequencies were compared across modalities to assess measurement reliability and consistency and to characterize the normal distribution of tremor frequencies in this population.ResultsAmong 160 participants (median age, 70 years; interquartile range [IQR], 64-77 years; 90.6% female [n = 145]), the median frequency of EVT was consistently between 4 and 5 Hz across all 3 methods (perceptual, 4.8 Hz [IQR, 4.4-5.5 Hz]; computerized peak detection, 4.6 Hz [IQR, 4.2-5.0 Hz]; and laryngeal electromyography, 4.3 Hz [IQR, 3.8-5.0 Hz]). The mean in-person differences between each measurement method were not clinically meaningful (range, 0.1-0.5 Hz). Including all interquartile ranges across measurement modalities, the normative tremor frequency range for EVT was 3.8 to 5.5 Hz.Conclusions and relevanceTo our knowledge, this is the largest study to date to characterize the normal frequency of tremor in patients with EVT. The normative frequency of EVT (range, 3.8-5.5 Hz) falls within a much narrower range than previously reported. Those whose frequency is outside this range may still have EVT but should be carefully evaluated for potential other causes of vocal tremor. Defining characteristics of EVT may aid appropriate diagnosis and improve understanding of this disease.

Project description:PurposeHealth literacy is a current Public Health priority in Portugal. The participation of well-informed patients in their care and shared decision making are essential, especially in chronic aggressive and debilitating pathologies such as recurrent or metastatic (R/M) Head and Neck Squamous Cell Carcinoma (HNSCC).AimsThis study aimed to characterize R/M HNSCC patients' and caregivers' information needs identified by healthcare professionals (HCPs).MethodsTwo online Focus Groups, one with only medical doctors and the other with other HCPs involved in the treatment of R/M HNSCC patients, were conducted, using a modified Metaplan, Lean or adapted PDCA methodology. The discussions were audio recorded in full and content analysis was performed using ATLAS.ti qualitative data analysis software.ResultsTopics addressed were diagnosis, treatment, quality of life, and global evaluation. In general, all experts agreed that only essential information should be cautiously given, according to patients' and caregivers' wishes. It was consensual that patients are given the necessary information to adhere to treatment. Two main barriers were identified: one barrier was associated with verbal communication due to the lack of health literacy of these patients, and the other barrier regarded healthcare access. It was also considered important to remind patients of the daily and social activities that they could and should maintain, as well as providing sufficient social resources and problem-solving training to caregivers.ConclusionsThis qualitative study highlights the complexity of R/M HNSCC patients' care. Immediate availability of psychologists and psychiatrists should be implemented in all centers that treat HNSCC patients. The differences found between the physicians' Focus Group and other HCPs' Focus Group in some of the addressed topics emphasize the importance of a multidisciplinary and holistic approach, in a biomedical model integrated with a biopsychosocial model.

Dataset Information

Head and voice tremor improving with immunotherapy in an anti-NF155 positive CIDP patient.

Publications

Head and voice tremor improving with immunotherapy in an anti-NF155 positive CIDP patient.

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