This scale is used when upper limb surgery is planned, as referred to in the International Classification for hand surgery in tetraplegic patients.A lesional segment (or an injured metamere) consists of denervated corresponding muscles. The lower motor neuron (LMN) of these muscles is damaged. These muscles are hypotonic, atrophic and have no spontaneous contraction. The existence of joint contractures should be monitored.Below the level of the injured metamere, there is an injured sublesional segment with the intact lower motor neuron, which means that medullary reflexes are present, but the upper cortical control is lost. These muscles show some increase in tone when elongated and sometimes spasticity, the trophicity is good. Incomplete spinal-cord lesions
Incomplete spinal cord injuries result in varied post injury presentations. There are three main syndromes described, depending on the exact site and extent of the lesion. Central cord syndrome: most of the cord lesion is in the gray matter of the spinal cord, sometimes the lesion continues in the white matter. Brown-Séquard syndrome: hemisection of the spinal cord. Anterior cord syndrome: a lesion of the anterior horns and the anterolateral tracts, with a possible division of the anterior spinal artery.For most patients with ASIA A (complete) tetraplegia, ASIA B (incomplete) tetraplegia and ASIA C (incomplete) tetraplegia, the International Classification level of the patient can be established without great difficulty. The surgical procedures according to the International Classification level can be performed.
Renpennings syndrome is a neurodevelopmental disorder recognised in males that causes intellectual disability, mild growth retardation with examples in the testes and head, and a somewhat short stature. The condition only affects males, starting at birth. Presentation
People with Renpennings typically begin learning language at an ordinary pace, but by the age of 3–4 they experience a regression in mental and physical development, such as mild low muscle tone resulting in elongated faces and rapid loss in the normal growth of the head (microcephaly). Small testes and short stature are also known to commonly occur. Genetics
It is associated with mutations in the PQBP1 gene. The gene product is a polyglutamine-binding protein involved in transcription and pre-mRNA splicing. The gene itself is located on the short arm of the X chromosome (Xp11.23). The most common mutations causing this condition occur in exon 4. Diagnosis
This diagnosis may be suspected on clinical grounds but should be confirmed by sequencing the PQBP1 gene. Treatment
There is no specific or curative treatment for this condition at present. Management is supportive

Epidemiology
This condition normally only occurs in males but a case in a female has been reported. History
This condition was first characterized in 1962. and later described by Hans Renpenning in 1963 after he documented these traits on many children in one family alone. See also
Lujan-Fryns syndrome
Fragile x syndrome

References


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