Familial spastic paraplegia or Strumpell-Lorrain disease

Familial spastic paraplegia or Strumpell-Lorrain disease
Familial spastic paraplegia is a eredofamiliala disease. Pathological, the bark of the motor area 4 Betz cells shows a progressive degeneration. Lesions in layers III and V of the crust are similar to lesions of Alzheimer's fibril type. The number of neurons decreases and degenerative lesions observed pyramidal tract. There are also less significant damage in cerebellar beam directly into the beam and column Golla Clarke.

 
Clinical

 
Clinically, the disease starts around age 18-20 years, but they are also described cases in which symptoms began under the age of one year. At first, symptoms are dominated by fatigue can be relieved by rest.

 
As time goes by there legs paresis, spastic paresis with character and attitude in the extension. Besides there are also other signs of pyramidal spasticity: tendon reflexes are exaggerated, there is a clonus of the leg and kneecap, and Babinski sign present.

 
Patients have a characteristic gait: the legs are exaggerated extension, leg weight is lifted off the ground, the knees tend to be close, toes are stuck, and the heels are apart. Trunk lean during gait previously appeared tilted gait.
Because spasticity is standing a foot varus equine form. Upper limbs are less affected than lower ones.
Sphincter disorders generally appear in advanced stages of disease.

 
The disease has a slow evolution. At the onset, symptoms appear only temporarily. Later spastic signs become permanent. Disease progression is very slow, sometimes between 20 and 30 years. Arm muscles and respiratory muscles is very little affected. Very rarely harm patients and other regions of the nervous system, including peripheral motor neuron involving amiotrofiilor appearance, mental retardation, optic atrophy, sensory neuropathy and skin thickening. Death occurs by intercurrent disease.

 
Differential Diagnosis

 
For a typical family forms the diagnosis is simple. For nefamiliale forms, the diagnosis is based on early onset and slowly progressive. The differential diagnosis is made with: - Multiple sclerosis - has a similar clinical picture with spastic paraplegia family, but in this case the disease is much faster and flare-ups. Analysis of cerebrospinal fluid is observed a high level of albumin. - Meningiomas parasagitale - this disease is developing faster and motor any sign of irritation of the lower limbs, signs of intracranial hypertension and disorders of sensitivity in the legs. - Various transverse myelitis, spinal cord compression or bone tumor, syphilitic endarterita can give a similar clinical picture, but in these cases is not so easily syndrome pyramidal spastic paraplegia that family. - Poisoning Latirus sativus, a plant with beans in the same botanical family, which causes a spastic papaplegie, but with rapid onset.

 
Treatment

 
There are currently no specific treatment. You should avoid burnout and alcoholic excesses. To decrease muscle hypertonia can recommend bathrooms transcerebromedulare ionization of calcium, or scopolamine injections relaxil. Treatment may sometimes be necessary and that tenotomii operators and use of orthopedic footwear is sometimes useful. Most people get to go another 30 years or more after onset disorders.