HEREDITARY SPASTIC PARAPLEGIA: SPORADIC CASES STUDY
Li M, Zhang J-J
The First Teaching Hospital of Beijing University,
Beijing, China
Objective: To
summarize the manifestations and characteristics of hereditary spastic
paraplegia in sporadic cases.
Methods:
The clinical data of eight children with
sporadic hereditary spastic paraplegia (HSP) were collected and six of them
were followed up using questionnaire.
Results: The
age at the onset was from 1.5 to 11 year old with the mean age of four and
half year old. Two patients were classified as simple form of HSP and 6
patients were complicated form. Except for the common progressive spastic
paraplegia, additional neurological abnormalities in the six cases of
complicated form of HSP included mental retardation, psuedo-bulbar palsy,
involvement of upper limbs, nystagmus and the more rapid deterioration of
paraplegia. The non-motor developmental delay in DDST in two young children
under 3 year old change from the simple form of HSP to the complicated form
later. Somatosensory evoked potentials (SEP) was detected in four cases,
and all of them revealed abnormal. The spinal MRI abnormality in one case
was firstly reported and accorded well with typical pathologic changes in
HSP.
Conclusion: The
non-motor developmental delay in DDST may predict the development of the
complicated form of HSP.