Little Foundation

Last updated
The William Little Foundation
Founded1990
FounderIan Dawson-Shepherd
Type Registered charity No. 803551
FocusUnderstanding the underlying causes of brain disorders, particularly cerebral palsy, and thereby develop means of preventing them
Location
Area served
International
Method Research funding, Public policy, and Education.
Key people
Lord Hameed, of Hampstead, Chairman
Website www.williamlittlefoundation.org

The William Little Foundation is a London-based charity operating internationally.

Contents

Named in honour of William Little, the English surgeon who described what became known as 'Little's disease', a spastic paralysis of both lower limbs which then became known as infantile cerebral palsy. The charity, founded in 1990 originally as The Little Foundation by the late Ian Dawson-Shepherd, [1] has as its primary object initiating and funding research [2] [3] [4] into the causes of neurodevelopmental disorders, [5] in particular the causes [6] and prevention [7] [8] of cerebral palsy, the most severe of these disorders which also includes autism, dyslexia, epilepsy, deafness, blindness and learning difficulties. It does this by supporting world-class basic research, hosting multi-disciplinary workshops and conferences, and providing a forum for the exchange of ideas and best practice across all parts of the UK’s cerebral palsy (CP) sector.

On 4 November 2009, Lord Hameed, the charity's president, tabled a motion in the House of Lords asking the Government what measures have been taken to prevent cerebral palsy which now costs the NHS £4 billion every year. [9] [10] The total annual cost of brain disorders (covering 19 groups of disorders) has been estimated to be €798 billion for the EU alone. [11]

In September 2020, the William Little Foundation published " Cerebral Palsy: causes and prevention ", a review of the state of CP research around the world, highlighting work that significantly enhanced our understanding CP but also identifying gaps in research activity where it was felt resources could be applied that would accelerate our ability to combat this devastating condition. Much of this concern centres on the commonly-held view of CP as a comparatively ‘rare’ condition, meaning that research investment is significantly less than for conditions with higher incidence and prevalence. While CP’s incidence is estimated to afflict between 1.5 and 4 babies in every 1,000, no one knows this for certain as there are no universally consistent means of diagnosing CP or reporting its incidence in either the developed or developing worlds.

This ‘rarity’ argument also ignores the disproportionate social cost that is CP’s legacy: the condition is lifelong and frequently involves 24-hour care for those affected. National social-/health-care budgets and those of medical insurance companies for those not supported by a national health service continue to be stretched by the need for ever-increasing provision: CP costs the UK alone well over £1.6 billion every year. Yet the amount invested annually on research by government and other charities is worryingly small: the figure for the UK is less than £5 million, 0.23% of research funding. The UK is not alone in this – the report also reveals a similar picture of cost : investment disparity globally. [12]

Research

The charity's most recent research projects include:

See also

Related Research Articles

Cerebral palsy Group of movement disorders that appear in early childhood

Cerebral palsy (CP) is a group of movement disorders that appear in early childhood. Signs and symptoms vary among people and over time, but include poor coordination, stiff muscles, weak muscles, and tremors. There may be problems with sensation, vision, hearing, and speaking. Often, babies with cerebral palsy do not roll over, sit, crawl or walk as early as other children of their age. Other symptoms include seizures and problems with thinking or reasoning, which each occur in about one-third of people with CP. While symptoms may get more noticeable over the first few years of life, underlying problems do not worsen over time.

Rett syndrome Genetic brain disorder

Rett syndrome (RTT) is a genetic disorder that typically becomes apparent after 6–18 months of age in females. Symptoms include impairments in language and coordination and repetitive movements. Those affected often have slower growth, difficulty walking, and a smaller head size. Complications of Rett syndrome can include seizures, scoliosis, and sleeping problems. The severity of the condition is variable.

Porencephaly is an extremely rare cephalic disorder involving encephalomalacia. It is a neurological disorder of the central nervous system characterized by cysts or cavities within the cerebral hemisphere. Porencephaly was termed by Heschl in 1859 to describe a cavity in the human brain. Derived from Greek roots, the word porencephaly means 'holes in the brain'. The cysts and cavities are more likely to be the result of destructive (encephaloclastic) cause, but can also be from abnormal development (malformative), direct damage, inflammation, or hemorrhage. The cysts and cavities cause a wide range of physiological, physical, and neurological symptoms. Depending on the patient, this disorder may cause only minor neurological problems, without any disruption of intelligence, while others may be severely disabled or die before the second decade of their lives. However, this disorder is far more common within infants, and porencephaly can occur both before or after birth.

Athetosis Medical condition

Athetosis is a symptom characterized by slow, involuntary, convoluted, writhing movements of the fingers, hands, toes, and feet and in some cases, arms, legs, neck and tongue. Movements typical of athetosis are sometimes called athetoid movements. Lesions to the brain are most often the direct cause of the symptoms, particularly to the corpus striatum. This symptom does not occur alone and is often accompanied by the symptoms of cerebral palsy, as it is often a result of this physical disability. Treatments for athetosis are not very effective, and in most cases are simply aimed at managing the uncontrollable movement, rather than the cause itself.

Sturge–Weber syndrome Medical condition

Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, intellectual disability, and ipsilateral leptomeningeal angioma. Sturge–Weber syndrome can be classified into three different types. Type 1 includes facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal lesions. Normally, only one side of the brain is affected. This type is the most common. Type 2 involvement includes a facial angioma with a possibility of glaucoma developing. There is no evidence of brain involvement. Symptoms can show at any time beyond the initial diagnosis of the facial angioma. The symptoms can include glaucoma, cerebral blood flow abnormalities and headaches. More research is needed on this type of Sturge–Weber syndrome. Type 3 has leptomeningeal angioma involvement exclusively. The facial angioma is absent and glaucoma rarely occurs. This type is only diagnosed via brain scan.

Periventricular leukomalacia Degeneration of white matter near the lateral ventricles of the brain

Periventricular leukomalacia (PVL) is a form of white-matter brain injury, characterized by the necrosis of white matter near the lateral ventricles. It can affect newborns and fetuses; premature infants are at the greatest risk of neonatal encephalopathy which may lead to this condition. Affected individuals generally exhibit motor control problems or other developmental delays, and they often develop cerebral palsy or epilepsy later in life. The white matter in preterm born children is particularly vulnerable during the third trimester of pregnancy when white matter developing takes place and the myelination process starts around 30 weeks of gestational age.

Intrauterine hypoxia Medical condition

Intrauterine hypoxia occurs when the fetus is deprived of an adequate supply of oxygen. It may be due to a variety of reasons such as prolapse or occlusion of the umbilical cord, placental infarction, maternal diabetes and maternal smoking. Intrauterine growth restriction may cause or be the result of hypoxia. Intrauterine hypoxia can cause cellular damage that occurs within the central nervous system. This results in an increased mortality rate, including an increased risk of sudden infant death syndrome (SIDS). Oxygen deprivation in the fetus and neonate have been implicated as either a primary or as a contributing risk factor in numerous neurological and neuropsychiatric disorders such as epilepsy, attention deficit hyperactivity disorder, eating disorders and cerebral palsy.

Epileptic spasms is an uncommon-to-rare epileptic disorder in infants, children and adults. One of the other names of the disorder, West syndrome, is in memory of the English physician, William James West (1793–1848), who first described it in an article published in The Lancet in 1841. The original case actually described his own son, James Edwin West (1840–1860). Other names for it are "generalized flexion epilepsy", "infantile epileptic encephalopathy", "infantile myoclonic encephalopathy", "jackknife convulsions", "massive myoclonia" and "Salaam spasms". The term "infantile spasms" can be used to describe the specific seizure manifestation in the syndrome, but is also used as a synonym for the syndrome itself. West syndrome in modern usage is the triad of infantile spasms, a pathognomonic EEG pattern, and developmental regression – although the international definition requires only two out of these three elements.

The Institutes for The Achievement of Human Potential (IAHP), founded in 1955 by Glenn Doman and Carl Delacato, provide literature on and teaches a controversial patterning therapy, which the Institutes promote as improving the "neurologic organization" of "brain injured" and mentally impaired children through a variety of programs, including diet and exercise. The Institutes also provides extensive early-learning programs for "well" children, including programs focused on reading, mathematics, language, and physical fitness. It is headquartered in Philadelphia, Pennsylvania with offices and programs offered in several other countries. Pattern therapy for patients with neuromuscular disorders was first developed by neurosurgeon Temple Fay in the 1940s. Patterning has been widely criticized and multiple studies have found the therapy ineffective.

Spastic diplegia Medical condition

Spastic diplegia is a form of cerebral palsy (CP) that is a chronic neuromuscular condition of hypertonia and spasticity—manifested as an especially high and constant "tightness" or "stiffness"—in the muscles of the lower extremities of the human body, usually those of the legs, hips and pelvis. Doctor William John Little's first recorded encounter with cerebral palsy is reported to have been among children who displayed signs of spastic diplegia.

Ohtahara syndrome (OS), also known as early infantile epileptic encephalopathy (EIEE) is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life, and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG). It is an extremely debilitating progressive neurological disorder, involving intractable seizures and severe intellectual disabilities. No single cause has been identified, although in many cases structural brain damage is present.

Dyskinetic cerebral palsy (DCP) is a subtype of cerebral palsy (CP) and is characterized by impaired muscle tone regulation, coordination and movement control. Dystonia and choreoathetosis are the two most dominant movement disorders in patients with DCP.

Athetoid cerebral palsy Type of cerebral palsy associated with basal ganglia damage

Athetoid cerebral palsy, or dyskinetic cerebral palsy, is a type of cerebral palsy primarily associated with damage, like other forms of CP, to the basal ganglia in the form of lesions that occur during brain development due to bilirubin encephalopathy and hypoxic–ischemic brain injury. Unlike spastic or ataxic cerebral palsies, ADCP is characterized by both hypertonia and hypotonia, due to the affected individual's inability to control muscle tone. Clinical diagnosis of ADCP typically occurs within 18 months of birth and is primarily based upon motor function and neuroimaging techniques. While there are no cures for ADCP, some drug therapies as well as speech, occupational therapy, and physical therapy have shown capacity for treating the symptoms.

Ataxic cerebral palsy Medical condition

Ataxic cerebral palsy is clinically in approximately 5–10% of all cases of cerebral palsy, making it the least frequent form of cerebral palsy diagnosed. Ataxic cerebral palsy is caused by damage to cerebellar structures, differentiating it from the other two forms of cerebral palsy, which are spastic cerebral palsy and dyskinetic cerebral palsy.

Spastic cerebral palsy Cerebral palsy characterized by high muscle tone

Spastic cerebral palsy is the type of cerebral palsy characterized by spasticity or high muscle tone often resulting in stiff, jerky movements. Cases of spastic CP are further classified according to the part or parts of the body that are most affected. Such classifications include spastic diplegia, spastic hemiplegia, spastic quadriplegia, and in cases of single limb involvement, spastic monoplegia.

Spastic hemiplegia Medical condition

Spastic hemiplegia is a neuromuscular condition of spasticity that results in the muscles on one side of the body being in a constant state of contraction. It is the "one-sided version" of spastic diplegia. It falls under the mobility impairment umbrella of cerebral palsy. About 20–30% of people with cerebral palsy have spastic hemiplegia. Due to brain or nerve damage, the brain is constantly sending action potentials to the neuromuscular junctions on the affected side of the body. Similar to strokes, damage on the left side of the brain affects the right side of the body and damage on the right side of the brain affects the left side of the body. Other side can be effected for lesser extent. The affected side of the body is rigid, weak and has low functional abilities. In most cases, the upper extremity is much more affected than the lower extremity. This could be due to preference of hand usage during early development. If both arms are affected, the condition is referred to as double hemiplegia. Some patients with spastic hemiplegia only experience minor impairments, where in severe cases one side of the body could be completely paralyzed. The severity of spastic hemiplegia is dependent upon the degree of the brain or nerve damage.

Ulegyria Type of cortical scarring deep in the sulci

Ulegyria is a diagnosis used to describe a specific type of cortical scarring in the deep regions of the sulcus that leads to distortion of the gyri. Ulegyria is identified by its characteristic "mushroom-shaped" gyri, in which scarring causes shrinkage and atrophy in the deep sulcal regions while the surface gyri are spared. This condition is most often caused by hypoxic-ischemic brain injury in the perinatal period. The effects of ulegyria can range in severity, although it is most commonly associated with cerebral palsy, mental retardation and epilepsy. N.C. Bresler was the first to view ulegyria in 1899 and described this abnormal morphology in the brain as “mushroom-gyri." Although ulegyria was first identified in 1899, there is still limited information known or reported about the condition.

General movements assessment

A general movements assessment is a type of medical assessment used in the diagnosis of cerebral palsy, and is particularly used to follow up high-risk neonatal cases. The general movements assessment involves measuring movements that occur spontaneously among those less than four months of age and appears to be most accurate test for the condition.

Perinatal stroke is a disease where an infant has a stroke between the 140th day of the gestation period and the 28th postpartum day, affecting up to 1 in 2300 live births. This disease is further divided into three subgroups, namely neonatal arterial ischemic stroke, neonatal cerebral sinovenous ischemic stroke, and presumed perinatal stroke. Several risk factors contribute to perinatal stroke including birth trauma, placental abruption, infections, and the mother's health. Detection and diagnosis of perinatal stroke are often delayed due to prenatal onset or inadequacy of neonatal signs and symptoms. A child may be asymptomatic in the early stages of life and may develop common signs of perinatal stroke such as seizures, poor coordination, and speech delays as they get older. Diagnostic tests such as magnetic resonance imaging, electroencephalogram, and blood tests are conducted when doctors suspect the patients have developed signs of a perinatal stroke. The prognosis of this disease is associated with the severity and the development of the symptoms. This disease can be treated by anticoagulant and anticonvulsant drugs, surgical procedures, and therapeutic hypothermia, depending on the condition of the patient.

SYNGAP1-related intellectual disability is a monogenetic developmental and epileptic encephalopathy that affects the central nervous system. Symptoms include intellectual disability, epilepsy, autism, sensory processing deficits, hypotonia and unstable gait.

References

  1. Hargreaves, William (19 January 1996). "Obituary: Ian Dawson-Shepherd". The Independent. London. Retrieved 25 May 2010.
  2. "Brain project; Funded by the Little Foundation." The Times [London, England] 22 Nov. 1990
  3. "Research on handicaps; Letter." The Times [London, England] 30 Aug. 1991
  4. "Baby brain damage research launched." The Times [London, England] 30 Jan. 1992: p.5
  5. 'The potential role for arachidonic and docosahexaenoic acids in protection against some central nervous system injuries in preterm infants' M. A. Crawford, I. Golfetto, K. Ghebremeskel, Y. Min, T. Moodley, L. Poston, A. Phylactos, S. Cunnane and W. Schmidt. Lipids 2003. Vol.38, Iss.4;p.303-315
  6. Hack M., Flannery D.J., Schluchter M., Cartar L., Borawski E., Klein N.(2002) 'Outcomes in young adulthood for very-low-birth-weight infants'. N. Engl. J. Med. 346 (3): 149-57.
  7. Czeizel A.E.(2009) 'Periconceptional folic acid and multivitamin supplementation for the prevention of neural tube defects and other congenital abnormalities.' Birth Defects Res. A Clin. Mol. Teratol. 85 (4): 260-8.
  8. Brough L., Rees G.A., Crawford M.A., Morton R.H. and Dorman E.K.(2010) 'Effect of multiple-micronutrient supplementation on maternal nutrient status and infant birth weight and gestational age at birth in a low income, multi-ethnic population.' B. J. Nutr., 23 April: 1-9.
  9. "Archived copy". Archived from the original on 2011-07-15. Retrieved 2009-11-07.{{cite web}}: CS1 maint: archived copy as title (link)
  10. https://publications.parliament.uk/pa/ld200809/ldhansrd/text/91104-gc0003.htm#091104114000035 [ bare URL ]
  11. "Archived copy" (PDF). Archived from the original (PDF) on 2012-03-05. Retrieved 2012-05-20.{{cite web}}: CS1 maint: archived copy as title (link) 'Cost of Disorders of the Brain in Europe' European Neuropsychopharmacology (2011) 21, 718–779
  12. Sassine, AnnieBelle (6 October 2020). "Cerebral Palsy: causes and prevention" (PDF). William Little Foundation.
  13. Turk J, Bax M, Williams C, Amin P, Eriksson M, Gillberg C (April 2009). "Autism spectrum disorder in children with and without epilepsy: impact on social functioning and communication". Acta Paediatrica. 98 (4): 675–81. doi:10.1111/j.1651-2227.2008.01184.x. PMID   19173683. S2CID   27735286.
  14. Rosenbaum P, Paneth N, Leviton A, et al. (February 2007). "A report: the definition and classification of cerebral palsy April 2006". Developmental Medicine and Child Neurology. 49 (S109): 8–14. doi: 10.1111/j.1469-8749.2007.tb12610.x . PMID   17370477.
  15. Bax M, Tydeman C, Flodmark O (October 2006). "Clinical and MRI correlates of cerebral palsy: the European Cerebral Palsy Study". JAMA. 296 (13): 1602–8. doi: 10.1001/jama.296.13.1602 . PMID   17018805.