Ten percent of people infected with HTLV-1 will develop certain kinds of cancer, lung disease and chronic degenerative diseases such as adult T-cell leukemia/lymphoma, bronchiectasis and HTLV-1-associated myelopathy/tropical spastic paraparesis.
|
|
A missense mutation (K382N) in VPS37A protein has been shown to cause complex hereditary spastic paraparesis (cHSP).
|
|
Among the methods of diagnosing tropical spastic paraparesis are MRI (magnetic resonance imaging) and lumbar puncture (which may show lymphocytosis).
|
|
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. As the name suggests, it is most common in tropical regions, including the Caribbean. Blood transfusion products are screened for HTLV-1 antibodies, as a preventive measure.
|
|
Those affected may be presented with bone pain due to bone lysis, lumbar paraparesis, and a variety of neurological symptoms. Vertebrae fractures are also common.
|
|
KIF1A is associated with hereditary spastic paraparesis. The website KIF1A.org serves as a resource for patients and care-givers, and provides links to research efforts.
|
|
For several decades, the term tropical spastic paraparesis was used to describe a chronic and progressive clinical syndrome that affected adults living in equatorial areas of the world. This condition was initially thought to be associated with infectious agents (such as Treponema pertenue and Treponema pallidum, which cause inflammation of the central nervous system) and with chronic nutritional deficiencies (such as avitaminosis) or exposure to potentially toxic foods (such as bitter cassava). Tropical myeloneuropathies are classified as two separate syndromes: tropical ataxic neuropathy (TAN) and tropical spastic paraparesis (TSP). They are placed together because they are found in tropical countries, although tropical spastic paraparesis has occurred in temperate countries (e.g.
|
|
Immunoglobulin Treatment of TSP involves corticosteroids to help with inflammation. Though any success with corticosteroids is short-lived, with symptoms worsened as the dosage is reduced. A synthetic derivative, 17-alpha-ethinyltestosterone, can be used to treat Tropical spastic paraparesis, improvement in motor and bladder function was reported but not sustainable. Mogamulizumab, an anti-CCR4 IgG1 monoclonal antibody, is also being researched as a possible treatment for Tropical spastic paraparesis.
|
|
The prognosis for Tropical spastic paraparesis indicates some improvement in a percentage of cases due to immunosuppressive treatment. A higher percentage will eventually lose the ability to walk within a ten-year interval.
|
|
The clinical symptoms are strikingly similar to those of lathyrism and also similar to tropical spastic paraparesis and hereditary spastic paraparesis, only that the latter two disorders have a slow onset. Konzo is distinct from polio which is a flaccid paralysis and most often affects a person asymmetrically. Konzo is one of several tropical neuropathies. A distinct myeloneuropathy also associated to cyanogen intake from cassava is tropical ataxic neuropathy (TAN), as first described in parts of Nigeria by B. O. Osuntokun in 1968.
|
|
In Tropical spastic paraparesis, HTLV-1 shows elevated cellular acquired immune response as well as high production of proinflammatory cytokines. IFN overexpression has been demonstrated as well. Also the number of NK cells (CD56+ and CD16+) is diminished.
|
|
Slow decline in acuity is known to occur in late middle age in some families. In complicated cases of autosomal dominant optic atrophy, in addition to bilateral optic neuropathy, several other neurological signs of neurological involvement can be observed: peripheral neuropathy, deafness, cerebellar ataxia, spastic paraparesis, myopathy.
|
|
Tapioca starch is made from cassava root. If prepared incorrectly, cassava root can be highly toxic as it naturally contains linamarin, a cyanogenic glycoside, which upon ingestion will release cyanide in the gut. Prolonged ingestion can result in acute cyanide poisoning, a goiter, spastic paraparesis, or ataxic neuropathy.
|
|
Manni was born in Lahti, Finland in 1992. He was born with paraparesis which saw his ability to walk unaided deteriorated from a young age. He obtained a degree in physical education from Pajulahti Sports Institute in Nastola. His younger brother Tuomas is also a para-athlete, competing in triathlon events.
|
|
Older patients affected with the cerebral form will present with similar symptoms. Untreated, cerebral ALD is characterized by progressive demyelination leading to a vegetative state and death. Adult males with an adrenomyeloneuropathy presentation typically present initially with muscle stiffness, paraparesis and sexual dysfunction. All patients with clinically recognized ALD phenotypes are at risk for adrenal insufficiency.
|
|
Occasional blurred vision and/or speech difficulties typically clear during the first month, except in severely affected patients. Spasticity is present from the first day, without any initial phase of flaccidity. After the initial weeks of functional improvement, the spastic paraparesis remains stable for the rest of life. Some patients may suffer an abrupt aggravating episode, e.g.
|
|
Persons with HDLS can suffer from tremors, decreased body movement, unsteadiness (Parkinsonism, muscles on one side of the body in constant contraction (spastic hemiparesis), impairment in motor and sensory function in the lower extremities (paraparesis), paralysis resulting in partial or total loss of all extremities and torso (tetraparesis), and the lack of voluntary coordination of muscle movements (ataxia).
|
|
Hereditary spastic paraplegia (HSP) is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness (spasticity) and contraction in the lower limbs. HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord.
|
|
Human MMC-like lesions with similar neurological deficit were found in the control newborn lambs. In contrast, animals that underwent closure had near- normal neurological function and well-preserved cytoarchitecture of the covered spinal cord on histopathological examination. Despite mild paraparesis, they were able to stand, walk, perform demanding motor test and demonstrated no signs of incontinence. Furthermore, sensory function of the hind limbs was present clinically and confirmed electrophysiologically.
|
|
The onset of paralysis (spastic paraparesis) is sudden and symmetrical and affects the legs more than the arms. The resulting disability is permanent but does not progress. Typically, a patient is standing and walking on the balls of the feet with rigid legs and often with ankle clonus. Initially, most patients experience generalized weakness during the first days and are bedridden for some days or weeks before trying to walk.
|
|
Because of the close relationship between BLV and HTLV-I, the research on BLV is important. One can use the experience with BLV for understanding HTLV-I induced diseases like ATL (adult T-cell leukemia) and HAM/TSP (HTLV-1-associated myelopathy/Tropical spastic paraparesis)-like neurological disorders. A number of case-control studies have been conducted, but research into BLV-related diseases has not been as extensive as that conducted into other viral diseases.
|
|
The major feature of HSP is a length dependent axonal degeneration.Wharton SB, McDermott CJ, Grierson AJ, Wood JD, Gelsthorpe C, Ince PG, Shaw PJ (2003) The cellular and molecular pathology of the motor system in hereditary spastic paraparesis due to mutation of the spastin gene. J Neuropathol Exp Neurol 62:1166–1177 These include the crossed and uncrossed corticospinal tracts to the legs and fasciculus gracilis. The spinocerebellar tract is involved to a lesser extent.
|
|
Little was born at the Red Lion Inn in Whitechapel. His parents John and Hannah little were the inn's proprietors.George Bentley, ‘Little, William John (1810–1894)’, Oxford Dictionary of National Biography, Oxford University Press, 2004 accessed 9 Nov 2016 Little did not have any spasticity himself, but he suffered childhood poliomyelitis with residual left lower-extremity paraparesis, complicated by severe talipes. This undoubtedly sparked his special interest in lower- extremity mobility impairments, as well as his medical-orthopedic inclinations more generally.
|
|
Underlying medical conditions such as diabetes mellitus, chronic kidney disease, and cancer can worsen the long-term survival and disability of those who recover from infection. The most severe complication of melioidosis is encephalomyelitis. It can cause quadriparesis (muscle weakness in all the limbs), partial flaccid paraparesis (muscle weakness of both legs), or foot drop. For those with previous melioidosis-associated bone and joint infections, complications such as sinus infection, bone and joint deformities with limited range of motion can occur.
|
|
The muscles of the neck may also be affected, and about half experience involvement of the cranial nerves that supply the head and face; this may lead to weakness of the muscles of the face, swallowing difficulties and sometimes weakness of the eye muscles. In 8%, the weakness affects only the legs (paraplegia or paraparesis). Involvement of the muscles that control the bladder and anus is unusual. In total, about a third of people with Guillain–Barré syndrome continue to be able to walk.
|
|
Mutations in this gene cause biotin-responsive basal ganglia disease (BBGD); a recessive disorder manifested in childhood that progresses to chronic encephalopathy, dystonia, quadriparesis, and death if untreated. Patients with BBGD have bilateral necrosis in the head of the caudate nucleus and in the putamen. Administration of high doses of biotin in the early progression of the disorder eliminates pathological symptoms while delayed treatment results in residual paraparesis, mild mental retardation, or dystonia. Administration of thiamine is ineffective in the treatment of this disorder.
|
|
The main symptoms of Devic's disease are loss of vision and spinal cord function. Optic neuritis may manifest as visual impairment with decreased visual acuity, although visual field defects, or loss of color vision may occur in isolation or prior to formal loss of acuity. Spinal cord dysfunction can lead to muscle weakness, reduced sensation, or loss of bladder and bowel control. The typical patient has an acute and severe spastic weakness of the legs (paraparesis) or all four limbs (quadriparesis) with sensory signs, often accompanied by loss of bladder control.
|
|
Emboli to the eye can be seen by ophthalmoscopy and are known as plaques of Hollenhorst. Emboli to the spinal cord may cause paraparesis (decreased power in the legs) or cauda equina syndrome, a group of symptoms due to loss of function of the distal part of the spinal cord - loss of control over the bladder, rectum and skin sensation around the anus. If the blood supply to a single nerve is interrupted by an embolus, the result is loss of function in the muscles supplied by that nerve; this phenomenon is called a mononeuropathy.
|
|
Viruses accepted to cause human cancers include some genotypes of human papillomavirus, hepatitis B virus, hepatitis C virus, Epstein–Barr virus, Kaposi's sarcoma-associated herpesvirus and human T-lymphotropic virus. The most recently discovered human cancer virus is a polyomavirus (Merkel cell polyomavirus) that causes most cases of a rare form of skin cancer called Merkel cell carcinoma. Hepatitis viruses can develop into a chronic viral infection that leads to liver cancer. Infection by human T-lymphotropic virus can lead to tropical spastic paraparesis and adult T-cell leukaemia.
|
|
It is characterized by normal development in early childhood, followed by rapid degeneration to a vegetative state. The other forms of ALD vary in terms of onset and clinical severity, ranging from adrenal insufficiency to progressive paraparesis in early adulthood (this form of the disease is typically known as adrenomyeloneuropathy). ALD is caused by mutations in ABCD1, a gene located on the X chromosome that codes for ALD, a peroxisomal membrane transporter protein. The exact mechanism of the pathogenesis of the various forms of ALD is not known.
|
|
If the upper cervical segment of the spinal cord is involved, all four limbs may be affected and there is risk of respiratory failure – the phrenic nerve which is formed by the cervical spinal nerves C3, C4, and C5 innervates the main muscle of respiration, the diaphragm. Lesions of the lower cervical region (C5–T1) will cause a combination of upper and lower motor neuron signs in the upper limbs, and exclusively upper motor neuron signs in the lower limbs. Cervical lesions account for about 20% of cases. A lesion of the thoracic segment (T1–12) will produce upper motor neuron signs in the lower limbs, presenting as a spastic paraparesis.
|
|
In his description, he coined the term transverse myelitis to reflect the band-like thoracic area of altered sensation that patients reported. The term 'acute transverse myelopathy' has since emerged as an acceptable synonym for 'transverse myelitis', and the two terms are currently used interchangeably in the literature. The definition of transverse myelitis has also evolved over time. Bastian's initial description included few conclusive diagnostic criteria; by the 1980s, basic diagnostic criteria were established, including acutely developing paraparesis combined with bilateral spinal cord dysfunction over a period of <4 weeks and a well-defined upper sensory level, no evidence of spinal cord compression, and a stable, non-progressive course.
|
|
HTLV-II has not been clearly linked to any disease, but has been associated with several cases of myelopathy/tropical spastic paraparesis (HAM/TSP)- like neurological disease and may cause chronic lung problems. An impact on platelet count has been observed. In the 1980s, HTLV-2 was identified in a patient with an unidentified T cell lymphoproliferative disease that was described as having characteristics similar to the B cell disorder, hairy cell leukemia. HTLV-2 was identified in a second patient with a T cell lymphoproliferative disease; this patient later developed hairy cell leukemia, but HTLV-2 was not found in the hairy cell clones.
|
|
The recurrent artery of Heubner, Heubner's artery or medial striate artery is named after the German paediatrician Otto Heubner and is a branch of the anterior cerebral artery, most often arising from the A1-A2 junction (44%) or the proximal A2 segment (43%), or more rarely from the A1 segment. Its vascular territory is the anteromedial section of the caudate nucleus and the anterioinferior section of the internal capsule, as well as parts of the putamen and septal nuclei. In cases of obstructed flow in the Heubner's artery, the individual may experience infarction in those subcortical areas and thus hemiparesis. More proximal portions of the artery may cause spastic paraparesis and sensory loss contralateral to the lesioned side.
|
|
The human T-lymphotropic virus, human T-cell lymphotropic virus, or human T-cell leukemia-lymphoma virus (HTLV) family of viruses are a group of human retroviruses that are known to cause a type of cancer called adult T-cell leukemia/lymphoma and a demyelinating disease called HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). The HTLVs belong to a larger group of primate T-lymphotropic viruses (PTLVs). Members of this family that infect humans are called HTLVs, and the ones that infect Old World monkeys are called Simian T-lymphotropic viruses (STLVs). To date, four types of HTLVs (HTLV-1, HTLV-2, HTLV-3, and HTLV-4) and four types of STLVs (STLV-1, STLV-2, STLV-3, and STLV-5) have been identified.
|
|
DeBakey was first to perform cardiopulmonary bypass to repair the ascending aorta, using antegrade perfusion of the brachiocephalic artery. By the mid-1960s, at Baylor College of Medicine, DeBakey’s group began performing surgery on thoracoabdominal aortic aneurysms (TAAA), which presented formidable surgical challenges, often fraught with serious complications, such as paraplegia, paraparesis and renal failure. DeBakey protégé and vascular Surgeon, E. Stanley Crawford, in particular, began dedicating most of his time to TAAAs. In 1986, he classified TAAA open surgery cases into four types: Extent I, extending from the left subclavian artery to just below the renal artery; Extent II, from the left subclavian to below the renal artery; Extent III, from the sixth intercostal space to below the renal artery; and Extent IV, from the twelfth intercostal space to the iliac bifurcation (i.e.
|
|
HTLV-1 is an abbreviation for human T-cell lymphotropic virus type 1, also called human T-cell leukemia type 1, a virus that has been implicated in several kinds of diseases, including tropical spastic paraparesis, and as a virus cancer link for leukemia (see adult T-cell leukemia/lymphoma). HTLV-1 has six reported subtypes (subtypes A to F). The great majority of infections are caused by the cosmopolitan subtype A. HTLV was discovered by Robert Gallo and colleagues in 1980. Between 1 in 20 and 1 in 25 infected people are thought to develop cancer as a result of the virus. HTLV-1 infection is thought to spread only through dividing cells since reverse transcriptase generates proviral DNA from genomic viral RNA, and the provirus is integrated into the host genome by viral integrase after transmission.
|
|
In melanocytic cells RIPK5 gene expression may be regulated by MITF. Mutations in this gene have been associated with hereditary spastic paraplegia type 23.Lee JYW, Hsu CK, Michael M, Nanda A, Liu L, McMillan JR, Pourreyron C, Takeichi T, Tolar J, Reid E, Hayday T, Blumen SC, Abu-Mouch S, Straussberg R, Basel- Vanagaite L, Barhum Y, Zouabi Y, Al-Ajmi H, Huang HY, Lin TC, Akiyama M, Lee JYY, McLean WHI, Simpson MA, Parsons M, McGrath JA (2017) Large intragenic deletion in DSTYK underlies autosomal-recessive complicated spastic paraparesis, SPG23. Am J Hum Genet 100(2):364-370 "Diagram of HsInv0006 (orange bar) genomic region showing the effect of the inverted allele on the expression of neighboring genes in different tissues according to the GTEx data and the inversion tag SNP in Europeans associated to increased risk of Glaucoma" Giner-Delgado, Carla, et al.
|
|