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Acta Myol. 2008 October; 27(2): 45–48.
PMCID: PMC2858937

Clinical semiology of neuromuscular diseases (5)

After thenar eminence amyotrophy (Part 1), after hand weakness and wasting (Part 2), after arm and forearm amyotrophy (Part 3), after Bent spine syndrome (Part 4), this fifth chapter deals with several types of neck atrophies.

  1. Sternomastoid muscle atrophy is of course a prominent element of type I Myotonic Dystrophy (Steinert disease).
    It is also a not unfrequent sign of inclusion body myositis.
  2. Dropped head syndrome is different from bent spine syndrome.
    It is often secondary to neuromuscular diseases for instance motor neuron disease or myasthenia gravis or dermatomyositis.
  3. Rigid spine syndrome (Dubowitz syndrome) is sometimes a restricted form of arthrogryposis or a sign of Emery-Dreifuss syndrome.
  4. Painful torticollis is frequent, viral or a frigore.
    Spasmodic retrocollis or antecollis are rare substypes of spasmodic torticollis.
    Hysterical torticollis is different.
  5. Head lag in a baby is among the symptoms of floppy baby.
    Therefore several types of neck muscle weakness or wasting are observed.
    Some are hallmarks of a disease.
Figure 1
Sternomastoid muscle atrophy in a patient with myotonic dystrophy, Steinert disease. Atrophy is symmetrical and bilateral.
Figure 2
Two cases of inclusion body myositis. Sternomastoid muscle atrophy is a not uncommon sign of the disease.
Figure 3Figure 3
Dropped head syndrome.
Figure 4
A cause of dropped head syndrome in a motor neuron disease.
Figure 5
Rigid spine syndrome in a case of congenital arthrogryposis. No contact possible between chin and thorax.
Figure 6
Rigid spine syndrome in a case of Emery-Dreifuss myopathy with humeroperoneal weakness and atrial paralysis.
Figure 7
Paintful torticollis “a frigore”. Left trapezius muscle contracture.
Figure 8
Hysterical torticollis.
Figure 9
Spasmodic retrocollis.
Figure 10
Spasmodic antecollis.
Figure 11Figure 11
Head lag. Hypotonia in a floppy baby.
Figure 12
Neck muscle weakness in a dermatomyositis: Pillow sign.
Figure 13
Another case of dermatomyositis with neck muscle weakness.

Articles from Acta Myologica are provided here courtesy of Pacini Editore