Which conditions are included in the differential diagnoses of hereditary spastic paraparesis (HSP)?

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

Answer

The main differential diagnosis for HSP is PLS. This distinction is important for genetic counseling of family members and for the patient’s prognosis, in that HSP generally carries a more favorable prognosis. [2] Other components of the differential diagnosis of HSP are similar to those of PLS. One study showed that in most patients with a sporadic adult-onset UMN syndrome, differentiation between HSP (with sporadic presentation) and PLS on the basis of clinical characteristics is unreliable and that genetic testing is therefore required. [2]

In addition, the differential diagnosis includes dopa-responsive dystonia and arginase deficiency. Given that both of these disorders are treatable, dopa-responsive dystonia should be excluded in a child with progressive gait disturbance and lower-extremity spasticity of unknown etiology, whereas arginase deficiency should be considered in a young child with progressive loss of developmental milestones and spasticity.


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