(Strumpell-Lorrain disease, spastic paraparesis)

Rare spinocerebellar hereditary degeneration (estimated prevalence: 2 to 10/100,000), genetically heterogeneous the mode of transmission of which is usually autosomal dominant but may be recessive or X-linked.

-        dominant forms: 8 genes including SPG3 gene on chromosome 14 and SPG4 gene on 2p24-p21.

-        recessive form: 4 genes including SPG7 gene

-        X-linked form: PLP gene or L1CAM gene on Xq-21-q22

Based on the clinical picture, there are:

-        pure forms: insidious onset typically between the age of 4 and 20 years and characterized by (sometimes minimal) walking difficulties; in addition: exaggerated tendon, bilateral Babinski reflex. After a few years appear hollow feet, deep sensitivity disorders, cerebellar signs, sphincteral problems, a distal amyotrophy and muscle aches. Pigmentary degeneration of the retina can be associated.

-        complex forms: clinical picture of pure forms combined with other neurologic involvements as, for example:

a) a distal muscular atrophy and degeneration of the retina: Kjellin syndrome

b) a peripheral neuropathy: hereditary sensorimotor polyneuropathy type V (HMSN V)

c) X-linked hydrocephalus

d) a distal muscular atrophy, pigmentary retinopathy, a mental deficit with microcephaly and congenital ichthyosis: Sjogren-Larsson syndrome (on chromosome 17p11.2).

Treatment is symptomatic: rehabilitation, antispasmodic.

Anesthetic implications:

avoid succinylcholine: a few cases of enhanced sensitivity to hypnotics (BIS) and non-depolarizing agents have been published; some cases of spinal and epidural blocks have been reported in obstetrics.

References : 

• McTiernan C, Haagenvik B. 
  Strümpell’s disease i a patient presenting for Cesarean section. 
  Can J Anesth 1999; 46: 6779-82.
• Thomas I, Thomas M, Scrutton M. 
  Spinal anaesthesia in a patient with hereditary spastic paraplegia: case report and review of the literature.
  Int J Obst Anesth 2006; 15: 254-6.
• Deruddre S, Marie M, Benhamou D.
  Subarachnoid anesthesia for cesarean delivery in a parturient with Strumpell-Lorrain disease.
  Anesth Analg 2006 ; 102 : 1910-1.
• Ponsonnard S, Damon A, Gueye EM.
  Anaesthesia and orphan disease : management of a case of Strumpell-Lorrain disease and review of the literature.
  Eur J Anaesthesiol 2017 ; 34 : 562-3.
• Yang PF, Links DJR, Petrovski J.
  Anesthetic considerations for Ivor-Lewis esophagectomy in a patient with hereditary spastic paraplegia: a case report.
  A&A Practice 2018; 11: 296-8.

Updated: December 2018