AS mimics
As ALS Mimics or ALS Mimic Syndromes , German ALS-like disease disorders are referred to, the early course of amyotrophic lateral sclerosis may resemble (ALS) strong and as differential diagnoses must be excluded. Such an ALS-like disease is behind about 10% of ALS diagnoses. This is important in everyday clinical practice because there is currently no therapy for ALS. The wrong diagnosis then leads to a potentially treatable disease being overlooked.
Recognized ALS mimics
The following diseases are recognized as ALS mimics:
- CIDP (chronic inflammatory demyelinating polyradiculoneuropathy)
- Multifocal motor neuropathy
- Sporadic inclusion body myositis
- Kennedy type spinobulbar muscular atrophy
- spinal muscular atrophy
- Neuromyotonia
- metabolic diseases ( adrenoleukodystrophy , metachromatic leukodystrophy , Tay-Sachs syndrome ) and cervical myelopathy
- Hereditary Motor and Sensitive Neuropathy (HMSN)
- Mitochondrial disease
Less rare ALS mimics are:
- Myasthenia gravis (especially since antibodies are often present in neuromyotonia, as in MG)
- Lambert-Eaton-Rooke Syndrome
- poliomyelitis
- Post poliomyelitis
- Heavy metal poisoning.
Separation into UMN and LMN
Some ALS mimics can already be excluded by their appearance. An ALS usually consists of an involvement of the upper (first) motor neuron (UMN - upper motor neuron) and the lower (second) motor neuron (LMN - lower motor neuron).
A defect in the first motor neuron leads to spasticity . A defect in the second motor neuron leads to atrophy (muscle wasting) and fasciculations . In the case of spasticity, it must first be clarified whether it is not due to other increases in tone . Myotonia or neuromyotonia can also act like spasticity. In the advanced stages, MRI is suitable here; the first motor neuron is involved through hypertense pyramidal tracts . At the same time, various ALS mimics (e.g. copper salt deposits in Wilson's disease) can be recognized.
literature
- E. Cortés-Vicente, J. Pradas, J. Marín-Lahoz, N. De Luna, J. Clarimón, J. Turon-Sans, E. Gelpí, J. Díaz-Manera, I. Illa, R. Rojas-Garcia : Early diagnosis of amyotrophic lateral sclerosis mimic syndromes: pros and cons of current clinical diagnostic criteria. In: Amyotrophic lateral sclerosis & frontotemporal degeneration. Volume 18, number 5-6, August 2017, pp. 333-340, doi : 10.1080 / 21678421.2017.1316408 , PMID 28440098 .
- M. Ghasemi: Amyotrophic lateral sclerosis mimic syndromes. In: Iranian journal of neurology. Volume 15, Number 2, April 2016, pp. 85-91, PMID 27326363 , PMC 4912674 (free full text) (review).
- BJ Traynor, MB Codd, B. Corr, C. Forde, E. Frost, O. Hardiman: Amyotrophic lateral sclerosis mimic syndromes: a population-based study. In: Archives of neurology. Volume 57, Number 1, January 2000, pp. 109-113, PMID 10634456 .
- Martin R. Turner, Kevin Talbot: Mimics and chameleons in motor neurone disease. In: Practical Neurology. Volume 13, number 3, 2013, pp. 153 ff., Doi : 10.1136 / practneurol-2013-000557 .
Individual evidence
- ↑ a b A. Hansel, J. Dorst, A. Rosenbohm, A. Hübers, A. Ludolph: ALS Mimics. In: Neurology International Open. 02, 2018, p. E60, doi : 10.1055 / s-0043-119960 .
- ^ LC Wijesekera, PN Leigh: Amyotrophic lateral sclerosis. In: Orphanet Journal of Rare Diseases. 4, 2009, p. 3, doi : 10.1186 / 1750-1172-4-3 .
- ↑ Cecilia Quarracino, María Constanza Segamarchi, Gabriel E. Rodríguez: Predictors of amyotrophic lateral sclerosis mimic syndrome . In: Acta Neurologica Belgica . tape 119 , no. 2 , June 1, 2019, ISSN 2240-2993 , p. 253-256 , doi : 10.1007 / s13760-019-01135-1 , PMID 30972662 .
- ↑ Jan Kruse et al: Diffusion disorders of the pyramidal tract in hereditary motor and sensory neuropathy (HMSN) type I and previously unknown BAG3 gene variant. Retrieved June 1, 2019 .
- ↑ Neuromuscular Disorders Affecting the Thorax: Amyotrophic Lateral Sclerosis. January 23, 2019. Retrieved May 30, 2019 (American English).