Hoffmann Syndrome
| Classification according to ICD-10 | |
|---|---|
| E03.9 | Hypothyroidism, unspecified myxedema nos |
| ICD-10 online (WHO version 2019) | |
The Hoffmann syndrome is a rare disorder of adulthood with a combination of hypothyroidism and an apparent muscle hypertrophy associated with painful spasms and Pseudomyotonia. It is also called adult hypothyroid myopathy .
The name refers to the first description in 1896 by the German neurologist Johann Hoffmann .
The disease should not be confused with Charcot-Marie-Tooth-Hoffmann syndrome .
root cause
The cause of muscular pseudohypertrophy is unknown, but it is believed to be a result of long-term hypothyroidism .
clinic
The disease only manifests itself in adulthood. Diagnostic criteria are:
- Long-standing untreated hypothyroidism, see hypothyroidism # Clinical picture .
- General muscle weakness without muscle atrophy
- Pseudomyotonic stiffness
- Slowed muscle reflexes
- Involvement of the peripheral nervous system
Investigation methods
Laboratory chemistry shows an increased creatine kinase activity in the blood serum , in the EMG no myotonic reaction can be detected.
Differential diagnosis
The Kocher-Debré-Semelaigne syndrome , which already occurs in children, but without painful spasticity and pseudomyotonia, must be distinguished.
therapy
Treatment consists of the administration of thyroid hormones , which enables complete normalization.
Individual evidence
- ↑ a b c d e B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9
- ^ J. Hoffmann: Another contribution to the theory of tetany. 1896 in: Deutsche Zeitschrift für Nervenheilkunde 9, pp. 278–290.
