Degenerative and hereditary myelopathy
One type of hereditary diseases, that cause patients spinal cord lesions syndromes, is Friedreich's ataxia. Friedreich's ataxia - a progressive hereditary autosomal recessive disease that causes the lower extremities’ and trunk's ataxia. Friedreich's ataxia is manifested in late childhood. In patients with Friedreich's ataxia are revealed:
- intention tremor,
- awkwardness in the hands,
- speech disorder (dysarthria).
Often in patients with hereditary myelopathy encountered kyphoscoliosis and strephopodia (pes cavus). On patient's examination revealed the absence of tendon reflexes (areflexia), abnormal Babinski sign and expressed impairments of vibration and muscle-joint sense. Also observed fragmented and mild forms of Friedreich's ataxia. They observed in conjunction with other syndromes:
- spastic paraparesis (Sgtrumpel-Laurent form),
- cerebellar cortical degeneration with ataxia,
- olivopontotserebellyarnoy atrophy (OPCA).
Anatomy of the spinal membranes and surrounding spinal canal structures.
In the presence of symmetric spastic paraparesis, not accompanied by sensitivity disorders in the patient, can be assumed amyotrophic lateral sclerosis (ALS, motor neuron disease). Amyotrophic lateral sclerosis (ALS) causes movement disorders, affecting the cortico-spinal, cortico-bulbar tract along with the spinal cord anterior horns neurons. Patient's muscle visible twitching (fasciculation) and signs of innervation absence on electromyography (EMG) study are signs of the motor neurons destruction in amyotrophic lateral sclerosis (ALS).
Spinal cord's subacute combined degeneration in vitamin B12 deficiency - a type of myelopathy, which causes patients motor and sensory disorders:
- progressive muscle spasm and atactic paraparesis with polyneuropathy,
- expressed distal paresthesias in the feet and hands.
Spinal cord's subacute combined degeneration in vitamin B12 deficiency is well treatable disease.
Spinal cord's subacute combined degeneration in vitamin B12 deficiency in patients must be differentiated from:
- cervical spondylosis,
- degenerative myelopathy with late-onset,
- spinal cord symmetric multiple sclerosis with late-onset.
In this condition brain, peripheral and optic nerves is also may be affected. The diagnosis is confirmed by blood plasma laboratory tests, when detected low levels of vitamin B12 and positive Schilling test. Expert opinion about the ability of folate or vitamin E deficiency in a patient's plasma lead to the development of a similar syndrome are contradictory. Sometimes, in the same patient can be detected B12 deficient and multiple sclerosis myelopathy simultaneously.
If you have any questions about the diagnosis or treatment of degenerative and hereditary myelopathy, you can specify them with our neurosurgeon or neurologist by phone: (499) 130-08-09
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