Motor problems in Parkinson’s disease: fluctuations, gait, balance and falls
Jeremy Playfer, John Hindle, Andrew Lees in Parkinson's Disease in the Older Patient, 2018
Most common tasks require frequent turns, and turning while walking is a potent source of akinetic blocks, leading to freezing episodes and falls. Disordered axial movement also shows up as difficulty in turning over in bed, and its presence appears to be related more to the duration of disease than the age of onset.48 Although difficulty in turning in bed is regarded as a characteristic feature of PD it is not diagnostic, as it can be found in 9% of healthy elderly subjects and in 38% of elderly patients without neurological disease attending a geriatric day hospital.49 Difficulty in turning over in bed is not an apraxia, but is due to bradykinesia and disruption of the normal limb and trunk synergies. Thus it can be regarded, along with disordered gait and loss of arm swing when walking, as another example of basal ganglia disease causing loss of sequencing of a well-practised, automatic movement. Difficulty in turning in bed is associated significantly with disturbed gait, postural instability, difficulty in rising from a chair, whole-body bradykinesia and axial rigidity. All of these axial motor impairments respond to levodopa.48
Hyperkinetic Movement Disorders
Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw in Hankey's Clinical Neurology, 2020
Metabolic: Thiamine responsive basal ganglia disease.Mitochondrial thiamine transporter deficiency.Lesch–Nyhan syndrome (LNS).Glucose transporter type 1 deficiency (GLUT1).
Leigh Syndrome (Encephalopathy)
Charles Theisler in Adjuvant Medical Care, 2023
Biotin: Specific therapy via biotin administration is appropriate for three forms of Leigh-like syndrome: biotin-thiamine-responsive basal ganglia diseasebiotinidase deficiencycoenzyme Q10 deficiency due to PDSS2 mutation4
Sense and nonsense concerning biotin interference in laboratory tests
Published in Acta Clinica Belgica, 2022
Alena Moerman, Joris R. Delanghe
Biotin is a water soluble vitamin/micronutrient and a coenzyme to 5 carboxylases. It is also covalently bound to lysine residues in histones. Because of this, it plays a role in the epigenetic regulation of genes, the structure of chromatin and cell signaling. Mammals cannot produce biotin and are consequently fully dependent on intake through the nutrition. Normal daily intake in western countries ranges from 35 to 70 µg per day, which lies above the daily recommended 5 to 35 µg. Our western intake pattern results in serum concentrations ranging from 0.12 to 0.36 nmol/L [4]. Rarely, people can be deficient in biotin. This shortage occurs in biotinidase deficiency (1/60089 living births) and severely malnourished children. Treatment of such a deficiency consists of 5 to 30 mg biotin per day. Biotin-thiamin-responsive basal ganglia disease is a condition that affects the nervous system. This is also treated with high dose biotin, namely 5 to 10 mg per day. In conclusion, it is unnecessary for healthy subjects to use biotin supplements. However, marketeers have made convenient use of the property of biotin to promote protein synthesis and thereby also keratin production. Supplements are available in all seizes and weights with concentrations ranging from 50 µg in multivitamin preparations to 20 mg in preparations specifically targeting growth and quality of hair and nails. Proof of this claims is however lacking, with only very little scientific evidence of any beneficial effect [5,6].
Effects of utterance rate and length on the spatiotemporal index in Parkinson’s disease
Published in International Journal of Speech-Language Pathology, 2020
Shin Ying Chu, Steven M. Barlow, Jaehoon Lee, Jingyan Wang
Most of the PD participants tested in the present study demonstrated normal speech intelligibility scores (>95% SIT scores), with 40% of them were judged within normal speech production (subjective perceptual scores). As basal ganglia disease advances, resources for neural compensation and motor reorganisation are progressively limited and accompanied by reductions in speech intelligibility. Future studies will benefit from a longitudinal and or cross-sectional design involving larger numbers of patients to classify the speech motor reorganisation patterns at different stages of PD to fully address issues relating speech intelligibility, UPDRS staging and quantitative measures of speech motor control. In addition, the current findings underscore the importance of examining individual differences within individuals with PD. For group comparison between variables, we simply pooled and contrasted test results with controls. Follow-up study will benefit from an examination of the differences related to pre- and post-Levodopa treatment in a repeated measures design which will also generate much-needed data of reliability of measurement techniques.
Related Knowledge Centers
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