2. Hereditary spastic paraplegia (HSP) is a large group of inherited neurologic disorders, in which the prominent feature is a progressive spastic paraparesis.
3. Dr. Str ü mpell first described hereditary forms of spastic paraplegia in 1883. HSP is group of genetic disorders , the modes of inheritance: autosomal dominant, autosomal recessive, Mitochondrial inheritance and x-linked recessive. Hereditary spastic paraplegia (HSP) is also called familial spastic paraparesis .
4. In Europe, the frequency of HSP is estimated to be 1-9 cases per 100,000 population.. HSP may occur at any age, from infancy through late adulthood (eg, 85 y). Most patients experience the onset of symptoms between the second and fourth decades of life.
5. Degeneration of the ends of the corticospinal tracts within the spinal cord. The ends of the longest fibers, which supply the lower extremities, are affected to a much greater extent than are the fibers to the upper body. Most people with HSP do not have symptoms in the hands or arms. PATHOPHYSIOLOGY
9. Paraplegin , an ATP-dependent proteolytic complex located at the mitochondrial inner membrane, which controls protein quality and regulates ribosome assembly.
10.
11. CLASSIFICATION Hereditary Spastic Paraplegias are classified Based on the symptoms (pure form versus complicated form); Based on their mode of inheritance (autosomal dominant, autosomal recessive or x-linked) Based on the patient’s age at onset.
58. Many patients with HSP may not get their required amount sleep, because of leg cramps or spasms or as a result of the frequent need to urinate during the night. Stress and depression
