Hereditary Spastic Paraparesis

 

The salient findings on were bilateral symmetrical spasticity in the lower limbs.

 

On inspection, there was minimal muscle atrophy. There may be pes cavus and hammer toes.

 

There was increased muscle tone symmetrically throughout the lower limbs, which was spastic in nature.

 

Weakness was difficult to assess secondary to spasticity but seemed reduced symmetrically.

 

Reflexes were brisk.

 

Coordination was not impaired.

 

There were minimal sensory deficits (may be reduced below knees sometimes with sharp stimuli and vibration).

 

Further examination revealed normal tone and strength in the upper limbs, and nil evidence of cranial nerve or bulbar dysfunction.

 

The findings are suggestive of a spastic paraparesis. Differentials include:

• Hereditary spastic paraparesis (caused by length dependent degeneration of the corticospinal tract) - usually minimal sensory deficits although there can be

• Hereditary ataxias with spasticity such as certain spinocerebellar ataxias and Friedreich’s ataxia

• Spinal cord lesions

  • Extrinsic

    • Degenerative/osteophytes/bulge

    • Compression from tumours or abscess

    • Trauma

  • Intrinsic

    • Vascular malformations or infarcts

    • Subacute combined degeneration (weakness, sensory ataxia, spasticity, paraesthesias, preserved pain/temperature)

  • Specific cord syndrome

    • Anterior (anterior spinal artery)

    • Brown Sequard

    • Central (pain/temperature and UMN weakness arm > legs)

    • Subacute

• Brain lesions

  • Cerebral palsy

  • Other - tumour, vascular, demyelinating

• Demyelinating disorders such as:

  • Progressive MS

  • Neuromyelitis optica

• Motor neuron disease

  • Including motor only form

 

What do you know about HSP?

It can be X-linked, autosomal dominant, or recessive.

​