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Definitions - Neurological Conditions - K


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Kearns-Sayre Syndrome -  (KSS) is a disease caused by a 5,000 base deletion in the mitochondrial DNA. Kearnes-Sayre syndrome starts before the age of 20. It is a rare genetic disease in that it can be heteroplasmic, that is, more than one genome can be in a cell at any given time. Unlike most mitochondrial diseases, it is not maternally inherited. Rather, it occurs sporadically.

Kennedy's Disease - (KD) or X-linked spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease associated with mutations of the androgen receptor (AR). Because of its endocrine manifestations related to the impairment of the AR, it can be viewed as a variation of the disorders of the androgen insensitivity syndrome (AIS). It is named after WR Kennedy, a neurologist who was among the first to describe this disease.

Kinsbourne syndrome - or Opsoclonus myoclonus syndrome (OMS) is a rare neurological disorder of unknown causes which appears to be the result of an autoimmune process involving the nervous system. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year. It affects 2 to 3% of children with neuroblastoma.

Kleine-Levin Syndrome - KLS, is a rare disorder characterized by the need for excessive amounts of sleep (hypersomnia), (e.g. up to 20 hours a day); excessive food intake (compulsive hyperphagia); and an abnormally uninhibited sexual drive. Adolescent males are the predominant victims of the disorder. Individual sufferers may often become irritable, lethargic, and/or apathetic. KLS patients may appear disoriented and report hallucinations. Symptoms are cyclical; with days to weeks (even up to months) of suffering interspersed by weeks or months (even up to years) symptom-free.

Klippel-Feil Syndrome - is a rare disorder characterized by the congenital fusion of any 2 of the 7 cervical vertebrae. It is caused by a failure in the normal segmentation or division of the cervical vertebrae during the early weeks of fetal development. The prognosis for most individuals with KFS is good if the disorder is treated early and appropriately. Activities that can injure the neck should be avoided. Anomalies associated with the syndrome can be fatal if not treated, or if found too late to be treatable.

Klippel-Trenaunay Syndrome (KTS) - (KTS) is a congenital circulatory disorder characterized by hemiangiomas (abnormal benign growths on the skin consisting of masses of blood vessels), arteriovenous abscesses, and varicose veins, usually on the limbs. The affected limbs may be enlarged and warmer than normal. Fused toes or fingers, or extra toes or fingers, may be present. Bleeding may occur, often as a result of a rectal or vaginal tumor. KTS is a progressive disorder, and complications may be life-threatening.

Klüver-Bucy Syndrome - is a behavioral disorder that occurs when both the right and left medial temporal lobes of the brain malfunction. The amygdala has been a particularly implicated brain region in the pathogenesis of this syndrome. Klüver-Bucy Syndrome causes individuals to put objects in their mouths and engage in inappropriate sexual behavior. Other symptoms may include visual agnosia (inability to visually recognize objects), loss of normal fear and anger responses, memory loss, distractibility, seizures, and dementia. The disorder may be associated with herpes encephalitis and trauma, which can result in brain damage.

Korsakoff's Amnesic Syndrome - (Korsakoff's psychosis, amnesic-confabulatory syndrome), is a degenerative brain disorder caused by the lack of thiamine (vitamin B1) in the brain. There are six major symptoms of Korsakoff's syndrome: anterograde and retrograde amnesia, or severe memory loss; confabulation, that is, invented memories which are then taken as true due to gaps in memory sometimes associated with blackouts; meager content in conversation; lack of insight, and apathy (the patients lose interest in things quickly and generally appear indifferent to change).

Krabbe Disease - (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare, often fatal degenerative disorder that affects the myelin sheath of the nervous system. This condition is inherited in an autosomal recessive pattern. Infants with Krabbe disease are normal at birth. Symptoms begin between the ages of 3 and 6 months with irritability, fevers, limb stiffness, seizures, feeding difficulties, vomiting, and slowing of mental and motor development. In the first stages of the disease, doctors often mistake the symptoms with those of cerebral palsy.

Kugelberg-Welander Disease - Spinal Muscular Atrophy (SMA) Types I, II, and III belong to a group of hereditary diseases that cause weakness and wasting of the voluntary muscles in the arms and legs of infants and children. The disorders are caused by an abnormal or missing gene known as the survival motor neuron gene (SMN1), which is responsible for the production of a protein essential to motor neurons. Without this protein, lower motor neurons in the spinal cord degenerate and die. The type of SMA (I, II, or III) is determined by the age of onset and the severity of symptoms.

Kuru - is a disease which affects the brain. It is endemic among the Fore tribe of Papua New Guinea and is universally fatal. It is characterized by headaches, joint pains and shaking of the limbs. It is believed to be caused by prions and is related to Creutzfeldt-Jakob disease. It is best known for the epidemic that occurred in Papua New Guinea in the middle of the twentieth century. Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. The hallmark of a TSE disease is misshapen protein molecules that clump together and accumulate in brain tissue. There were no treatments that could control or cure kuru, other than discouraging the practice of cannibalism. Currently, there are no cures or treatments for any of the other TSE diseases.

Alphabetical list of Neurological Disorder Definitions.

A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - R - S - T - V - W - X - Z


 

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