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Updated: Jun 15, 2026
08:16
Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
[Facioscapulohumeral muscular dystrophy].
J Wilbers1, R R Frants, B G M van Engelen
1Afdeling Neurologie van het Universitair Medisch Centrum St Radboud, Nijmegen.
Nederlands Tijdschrift Voor Tandheelkunde
|February 26, 2010
Summary
Facioscapulohumeral muscular dystrophy (FSHD) is a genetic disorder causing progressive muscle weakness. Early recognition by oral health providers is crucial due to facial symptoms.
Area of Science:
- Genetics
- Molecular Biology
- Neurology
Context:
- Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant genetic disorder.
- Characterized by progressive weakness in facial, shoulder, and upper arm muscles.
- Caused by a DNA contraction on chromosome 4, leading to chromatin relaxation and gene dysregulation.
Purpose:
- To highlight the role of oral health care providers in early FSHD recognition.
- To emphasize the need for adequate oral health care in FSHD patients.
- To inform about the genetic basis and clinical manifestations of FSHD.
Summary:
- FSHD involves progressive muscle weakness, often starting with asymmetric facial muscles.
- The genetic cause is a DNA contraction on chromosome 4, affecting chromatin structure.
- Oral health professionals can identify early symptoms like facial weakness.
Impact:
- Facilitates earlier diagnosis and intervention for FSHD.
- Improves management of oral health complications associated with facial weakness.
- Increases awareness among healthcare providers about FSHD's initial presentation.
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