<unk> L-SMA is characterized by severe hypotonia and areflexia with loss of anterior horn cells in the spinal cord (i.e., lower motor neurons). The disease course is similar to that in the most severe forms of classic autosomal recessive SMA caused by mutation of SMN1: SMA type 0 (SMA0) and SMA type I (SMA1). In SMA0, prenatal onset of weakness and poor intrauterine movement results in congenital contractures. In SMA1, motor skills regress before age six months; affected children are never able to sit independently. The weakness of<unk> L-SMA is often prenatal in onset, manifests as polyhydramnios and poor movement in utero that results in congenital contractures. Moreover, the weakness of<unk> L-SMA is progressive. Below is a list of known symptoms of<unk> L-SMA: * Face : * Myopathic Facies * Facial weakness * Mouth : * Tongue fasciculations * Respiratory : * Insufficiency due to muscle weakness * Chest : * Chest deformities * Genitourinary : * External Genitalia (Male) * Hypospadias * Internal Genitalia (Male) * Cryptorchidism * Skeletal : * Arthrogryposis * Multiple joint contractures * Bone fractures (at birth and postnatal) * Skull- * Dysmorphic Skull * Hands- * Digital contractures Note: Clinical diagnosis of<unk>  should be considered for children who exhibit the following: * Evidence of degeneration and loss of anterior horn cells in the spinal cord and brain stem * Normal SMN1 molecular genetic testing to rule out autosomal recessive spinal muscular atrophy * Male gender in a simplex case or<unk><unk>  pattern of inheritance in families with more than one affected individual
X-linked spinal muscular atrophy type 2
Jokela type spinal muscular atrophy
Fukuyama congenital muscular dystrophy
Distal spinal muscular atrophy type 2
Ullrich congenital muscular dystrophy
Distal spinal muscular atrophy type 1
Madras motor neuron disease
Becker muscular dystrophy