tropical spastic paraparesis

What is Tropical Spastic Paraparesis?

Tropical spastic paraparesis (TSP) is a chronic and progressive disease of the nervous system that affects adults living in equatorial areas of the world. It is characterized by weakness, muscle spasms, and sensory disturbance, resulting in paraparesis, which is weakness of the legs.

Symptoms

The symptoms of TSP include:

• Progressive weakness of the legs
• Increased muscle tone
• Extensor plantar responses (a reflex that indicates nerve damage)
• Sensory signs, such as numbness or tingling sensations
• Optic nerve involvement, leading to vision problems

Prevalence and Demographics

TSP is most common in tropical regions, including the Caribbean. It affects adults more frequently than children, with a higher incidence in women than men.

Causes and Associations

TSP is associated with the human T-lymphotropic virus 1 (HTLV-1). The exact cause of the disease is not fully understood, but it is believed to be an immune-mediated disorder triggered by the HTLV-1 virus.

References

• [1] The term "tropical spastic paraparesis" has been used for several decades to describe this condition.
• [2] TSP causes weakness, muscle spasms, and sensory disturbance in adults living in equatorial areas of the world.
• [3] The disease is characterized by progressive weakness of the legs, increased muscle tone, and extensor plantar responses.
• [4] TSP affects adults more frequently than children, with a higher incidence in women than men.
• [5] The condition is associated with the human T-lymphotropic virus 1 (HTLV-1).
• [6] HTLV-1–associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a slowly progressive viral immune-mediated disorder of the spinal cord caused by the HTLV-1 virus.
• [7] TSP causes spastic weakness of both legs and is more frequently reported in women than in men.
• [8] The disease usually presents in adulthood with slowly progressive spastic paraparesis of the lower limbs, bladder and bowel dysfunction, and sensory disturbance.

Symptoms of Tropical Spastic Paraparesis (TSP)

Tropical spastic paraparesis, also known as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP), is a chronic and progressive disease that affects the nervous system. The symptoms of TSP can vary from person to person but often include:

• Gradually developing spastic weakness in both legs: This is one of the most common symptoms of TSP, where the muscles in the legs become weak and stiff.
• Extensor plantar responses: This refers to a reflexive response in the feet, where the toes are extended or "locked" when the sole of the foot is stimulated.
• Bilateral symmetric loss of position and vibratory sensation in the feet: People with TSP may experience numbness or tingling sensations in both feet, which can be symmetrical (i.e., affecting both sides equally).
• Absent Achilles tendon reflexes: The Achilles tendon reflex is a reflexive response that occurs when the calf muscles are stimulated. In people with TSP, this reflex may be absent.
• Slowly progressive weakness and spasticity of one or both legs: As the disease progresses, people with TSP may experience increasing weakness and stiffness in their legs.
• Exaggerated reflexes: People with TSP may have exaggerated reflexes in their legs, which can be a sign of nerve damage.

These symptoms can begin long after the original infection, sometimes decades after. The progression of the disease can vary from person to person, but it is often slow and gradual.

References:

• [4] - Tropical spastic paraparesis (TSP), is a medical condition that causes weakness, muscle spasms, and sensory disturbance by human T-lymphotropic virus resulting in paraparesis, weakness of the legs.
• [11] - HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a progressive disease of the nervous system that affects less than 2% of people with HTLV-1 infection. Signs and symptoms vary but may include slowly progressive weakness and spasticity of one or both legs, exaggerated reflexes, muscle contractions in the ankle, and lower ...
• [15] - Signs and symptoms vary but may include slowly progressive weakness and spasticity of one or both legs, exaggerated reflexes, muscle contractions in the ...

Diagnostic Tests for Tropical Spastic Paraparesis

Tropical spastic paraparesis (TSP), also known as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP), is a chronic and progressive disease of the nervous system. Diagnosing TSP can be challenging, but various tests can help confirm the condition.

Blood Tests

• Blood tests are used to detect the presence of human T-lymphotropic virus 1 (HTLV-1) antibodies in the blood [3][9].
• These tests can also measure the level of HTLV-1 proviral load, which is the amount of integrated HTLV provirus in the body [5].

Imaging Tests

• Magnetic Resonance Imaging (MRI) scans are used to visualize the spinal cord and detect any abnormalities [7][13].
• Computed Tomography (CT) scans may also be performed to rule out other conditions that can cause similar symptoms.

Lumbar Puncture

• A lumbar puncture, also known as a spinal tap, is a procedure where a sample of cerebrospinal fluid is collected from the lower back [3][13].
• This test can help detect the presence of HTLV-1 in the cerebrospinal fluid.

Other Tests

• Electromyography (EMG) and nerve conduction studies may be performed to assess muscle function and nerve damage.
• Ultrasound scans may also be used to evaluate the spinal cord and surrounding tissues.

It's worth noting that a definitive diagnosis of TSP can only be made by a healthcare professional based on a combination of clinical symptoms, laboratory tests, and imaging results [10][12].

References: [3] Context 3 [5] Context 5 [7] Context 7 [9] Context 9 [10] Context 10 [12] Context 12 [13] Context 13

Treatment Options for Tropical Spastic Paraparesis

Tropical spastic paraparesis (TSP), also known as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP), is a slowly progressive viral immune-mediated disorder of the spinal cord. While there is no cure for TSP, various treatment options are available to manage its symptoms and slow down disease progression.

Steroid Therapy

• High-dose pulsed methylprednisolone has been used as an induction therapy to reduce inflammation and improve symptoms [4].
• Low-dose oral prednisolone (5 mg) has been effective as maintenance therapy to prevent relapses [4].

Immunosuppressive Agents

• Cyclosporine has shown promise in treating new-onset TSP or chronic TSP with relapse, although its long-term safety profile is unclear [5].
• Mogamulizumab, an anti-CCR4 IgG1 monoclonal antibody, is being researched as a potential treatment for TSP. It works by reducing the number of immune cells that contribute to disease progression [7].

Muscle Relaxants and Urinary Dysfunction Treatments

• Lioresal or tizanidine may be prescribed to treat stiff and spastic muscles [6].
• Oxybutynin can help manage urinary dysfunction associated with TSP [6].

Other Treatment Options

• Danazol, an anabolic steroid, has been used in some cases to treat TSP, although its effectiveness is still being researched [9].
• Anticoagulants and decompressive surgery have also been explored as treatment options for TSP, but their use is more controversial and may not be recommended in all cases [8].

It's essential to note that each individual with TSP may respond differently to these treatment options. A healthcare professional should be consulted to determine the best course of treatment based on individual needs.

References: [4] High-dose pulsed methylprednisolone for induction therapy [5] Cyclosporine for treating new-onset or chronic TSP [6] Lioresal and tizanidine for muscle relaxation, oxybutynin for urinary dysfunction [7] Mogamulizumab as a potential treatment for TSP [8] Anticoagulants and decompressive surgery for TSP [9] Danazol for treating TSP

Understanding Differential Diagnosis in Tropical Spastic Paraparesis

Differential diagnosis refers to the process of identifying and ruling out other possible causes of a particular condition or symptom. In the context of tropical spastic paraparesis (TSP), differential diagnosis is crucial for accurate diagnosis and effective treatment.

Common Causes of Progressive Spastic Paraparesis

According to various studies [6, 10], some common causes of progressive spastic paraparesis that should be considered in the differential diagnosis of TSP include:

• Upper motor neuron lesions
• Spinal cord compression or injury
• Multiple sclerosis
• Amyotrophic lateral sclerosis (ALS)
• Hereditary spastic paraplegia (HSP)

Key Features to Consider

When considering a differential diagnosis for TSP, it is essential to look out for the following key features [4, 14]:

• Absence of radicular symptoms or sensory level
• Presence of spastic paraparesis as the dominant feature
• Slowly progressive disease course

Other Conditions to Rule Out

In addition to the above-mentioned conditions, other differential diagnoses that should be considered in TSP include [14]:

• Familial spastic paraplegia (FSP)
• Spinal muscular atrophy (SMA)

Importance of Accurate Diagnosis

Accurate diagnosis is critical for effective treatment and management of TSP. A comprehensive differential diagnosis approach can help clinicians identify the underlying cause of the condition, leading to more targeted and effective treatment strategies.

References:

[6] Yamano Y. HAM/TSP: a chronic inflammatory disease of the central nervous system. Journal of Neurology 2012;259(12):2511-2523.

[10] Mozhgani SH. High-throughput meta-analysis for identifying major genes involved in the pathogenesis of HAM/TSP. PLOS ONE 2019;14(5):e0217444.

[14] Román GC. Tropical spastic paraparesis (TSP) and its differential diagnoses. In: Handbook of Clinical Neurology, vol. 134, pp. 131-144.