Which conditions are included in the differential diagnoses of amyotrophic lateral sclerosis (ALS) (Lou Gehrig disease)?

Updated: Apr 28, 2014
  • Author: Hidehiro Takei, MD; Chief Editor: Adekunle M Adesina, MD, PhD  more...
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Answer

Answer

Conditions that should be considered in the differential diagnosis of amyotrophic lateral sclerosis (ALS) include the following:

  • Other neurodegenerative disorders, such as primary lateral sclerosis (PLS), hereditary spastic paraparesis (HSP), spinal muscular atrophy (SMA), and X-linked spinobulbar muscular atrophy (SBMA)

  • Peripheral neuropathies, such as multifocal motor neuropathy, an acquired immune-mediated demyelinating neuropathy with frequently elevated anti-GM1 antibody titers that usually responds to treatment with intravenous immunoglobulin (IVIg)

  • Myopathies, including inclusion body myositis, which is characterized by wasting and weakness of deep finger flexors and the quadriceps femoris with typical rimmed vacuoles on muscle biopsy

  • Postpolio syndrome (PPS)

  • Monomeric amyotrophy (also known as Hirayama disease), a structural cervical cord disorder due to dynamic compression of the cord that is seen only at neck flexion with forward displacement of the posterior dura; young males in the second and third decades of life are mainly affected, the onset is insidious, and predominantly unilateral upper-extremity weakness and atrophy occur, with no sensory or upper motor neuron (UMN) symptoms [65]

  • Conditions listed in the PLS differential diagnosis (see Differential Diagnosis)


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