Primary lateral sclerosis

Classification according to ICD-10
G12.2	Motor neuron disease
ICD-10 online (WHO version 2019)

The primary lateral sclerosis (PLS) is a very rare, to the motor neuron diseases associated with neurodegenerative disease with the main features of increasing muscle weakness of the skeletal muscles . Only the upper motor neuron is affected.

If it already manifests in childhood, it can be juvenile primary lateral sclerosis .

distribution

The frequency should be around 1 in 1,000,000, the causes have not yet been clarified. The mean age at the onset of the disease is 55 years.

Mutations in chromosome 4 locus p16 with an autosomal dominant inheritance were found in some patients .

Clinical manifestations

Clinical criteria are:

• Manifestation after the 5th decade of life, duration of illness over several years, at least 3 years (according to Pringle), 5 years (according to Stark)
• Gradually progressive, often asymmetrical spasticity mostly of the lower extremities, but sometimes also of the tongue or hands.
• Disturbance of the sense of balance , swallowing disorders
• Hyperreflexia with positive Babinski reflex

Differential diagnosis

The following are to be distinguished:

• Amyotrophic Lateral Sclerosis
• Low-malignant astrocytomas
• Dejerine-Thomas Syndrome
• Glioblastoma multiforme
• Hereditary forms of spastic paraplegia (HSP)
• Linamarine poisoning (Konzo)
• Lathyrism
• Lyme disease
• Meningiomas
• multiple sclerosis
• Multiple system atrophy
• Neurosyphilis
• Oligodendroglioma
• Progressive multifocal leukoencephalopathy
• Spinocerebellar ataxia
• Spondylosis deformans of the cervical spine
• Spinal cord tumors

literature

• JM Statland, RJ Barohn, MM Dimachkie, MK Floeter, H. Mitsumoto: Primary Lateral Sclerosis. In: Neurologic clinics. Vol. 33, No. 4, November 2015, pp. 749-760, doi: 10.1016 / j.ncl.2015.07.007 , PMID 26515619 , PMC 4628724 (free full text) (review).
• PP Urban, I. Wellach, C. Pohlmann: Slowly progressive dysarthrophonia in primary lateral sclerosis. In: The neurologist . Vol. 81, No. 8, August 2010, pp. 986-8, 990, doi: 10.1007 / s00115-010-3014-8 , PMID 20532474 .
• R. Heene, D. Kolander, H. Knisatschek: The chronic-progressive spinobulbar spasticity (primary lateral sclerosis). In: Advances in neurological psychiatry. Vol. 64, No. 5, May 1996, pp. 192-203, doi: 10.1055 / s-2007-996385 , PMID 8675141 (review).

Individual evidence

1. ↑ ^{a b c} Emedicine Medscape
2. ↑ Primary lateral sclerosis, adult, 1.  In: Online Mendelian Inheritance in Man . (English)
3. ^ CE Pringle, AJ Hudson, DG Munoz, JA Kiernan, WF Brown, GC Ebers: Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. In: Brain: a journal of neurology. Volume 115 (Pt 2), April 1992, pp. 495-520, PMID 1606479 .
4. ^ FM Stark, FP Moersch: Primary lateral sclerosis: a distinct clinical entity. In: Journal Nervous Mental Disease , Vol. 102, 1945, pp. 332-337.

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !