What is familial spastic paraparesis?
What is familial spastic paraparesis?
Definition. Hereditary spastic paraplegia (HSP), also called familial spastic paraparesis (FSP), refers to a group of inherited disorders that are characterized by progressive weakness and spasticity (stiffness) of the legs. Early in the disease course, there may be mild gait difficulties and stiffness.
Is Hereditary spastic paraplegia life threatening?
People with spastic paraplegia type 49 can develop recurrent episodes of severe weakness, hypotonia, and abnormal breathing, which can be life threatening.
What causes spastic paraparesis?
Most people with pure hereditary spastic paraplegia will have inherited a faulty gene from 1 of their parents. People with the complicated form of the condition will have usually inherited a faulty gene from both parents. The gene abnormality causes the long nerves in the spine to deteriorate.
Is there a cure for spastic paraparesis?
There are no specific treatments to prevent, slow, or reverse HSP. Treatment is symptomatic and supportive. Medications may be considered for spasticity and urinary urgency. Regular physical therapy is important for muscle strength and to preserve range of motion.
Is spastic paraparesis a disability?
HSP refers to a group of inherited neurologic disorders that cause progressive weakness and spasticity, or stiffness, in the lower extremities, mostly the leg and hip muscles. The long-term prognosis for people diagnosed with HSP varies: Some become very disabled, while others experience only mild disability.
Is spastic paraparesis painful?
Tightness in the legs and leg muscle spasm (often at night) are not uncommon. The consequences of abnormal walking pattern cause strain on the ankles, knees, hips, and back and often cause pain in these areas.
Is Hereditary spastic paraplegia painful?
The pain is not directly due to HSP itself but is instead often caused by muscle weakness and gait abnormalities resulting from HSP. As certain muscles become weaker, other muscles need to compensate for that weakness. Compensatory measures create an awkward gait that causes strain on many muscles and joints.
What is spastic paraparesis in MS?
Hereditary spastic paraparesis (HSP) is characterized clinically by progressive spasticity and weakness of the lower limbs and pathologically by retrograd axonal degeneration of the corticospinal tracts and posterior columns.
Is spastic paraplegia painful?
Is spasticity a disability?
Spasticity symptoms include continuous muscle stiffness, spasms and involuntary contractions, which can be painful. A person with spasticity may find it difficult to walk or perform certain tasks. Spasticity in children can result in growth problems, painful and deformed joints and disability.
Does spasticity get worse over time?
Keep moving. Muscle spasticity will tend to get worse the less you move. This is especially true if the muscles become weaker. Moving less can also contribute to developing contracture.
Is HSP similar to MS?
HSP shares several clinical features of the progressive phase in Multiple Sclerosis (MS) which is a chronic neurodegenerative disease characterized by multiple areas of central nervous system (CNS) inflammation, demyelination and axonal loss.
What are the symptoms of hereditary spastic paraparesis?
HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord.
What is the difference between paraparesis and spastic paraplegia?
“Paraparesis” indicates weakness in both legs of lesser severity than paraplegia. Although the disorder is typically referred to as hereditary spastic paraplegia the degree of weakness is variable and ranges from no weakness (full strength) to marked weakness (paraplegia).
Are there any synonyms for hereditary spastic paraplegia?
Synonyms of Hereditary Spastic Paraplegia 1 familial spastic paraplegia. 2 FSP. 3 HSP. 4 hereditary spastic paraparesis. 5 familial spastic paraparesis. 6 (more items)
What are the signs and symptoms of SPG11?
Signs and symptoms of SPG11 may include: Spasticity (progressive muscle stiffness) Paraplegia(eventual paralysis of the lower limbs) Numbness, tingling, or pain in the arms and legs. Disturbance in the nerves used for muscle movement.