61. Medicine On Earth: Amedeo Who's Who In Medicine JanFeb;27(1)60-5. Abstract Sridharan R, Radhakrishnan K, Ashok PP, Mousa ME Prevalenceand pattern of spinocerebellar degenerations in northeastern Libya. http://www.medicineonearth.com/display.php?id=2785&language=1

62. There Is One Chance In Four Of It Being A Normal Child, Two There are a number of less common spinocerebellar degenerations to be considered,such a Refsum’s disease, CharcotMarie-Tooth disease, and the Roussy-Levy http://www.fasi.ie/condition3.html

There is one chance in four of it being a normal child, two chances in four of it being a carrier, and one chance in four of it having FA. This is much the commonest way in which FA is inherited. Similarly it can be shown that, if an FA sufferer marries a normal person, all of the children will be carriers (ff+FF=Ff, Ff, Ff, Ff). If an FA sufferer marries an FA carrier, there is a 50/50 chance of the children being either carriers or sufferers of FA. Finally in the event of two FA sufferers marrying, all of the children will themselves suffer from FA (ff+ff=ff ,ff ,ff ,ff). Note that the FA carrier is apparently quite normal. If such a carrier is not a member of an FA family he/she will be unaware of the condition and therefore the carrier state may be passed on unknowingly for generation after generation (as in marriage 1). It is very important to note also that, if two FA carriers marry, the 1 in 4 chance of them having an FA child is true for each child born. In other words, should they have, say two FA children to start with, the chance of a third child having FA is unaffected - that is, the chance is still 1 in 4. It should be remembered that FA is only one of many recessive genes, that may in a double dose, i.e. inherited from both parents, cause some form of illness and that everyone carries some deleterious genes. Pathology 1. Cells of dorsal root and Clarke’s column Degeneration with gliosis. Hence sensory (dorsal root) and proprioceptive (Clarke’s column) loss. The Cells of Clarke’s column are involved also in adjusting muscle tone and synergising muscle movement. 2. Dorsal columns, spinocerebellar tracts, pyramidal tracts, and peripheral nerves Demyelination with gliosis, and reinforcement of effects indicated above. These changes in pyramidal (lateral corticospinal) tracts partly explain muscle weakness, disuse being the other element involved. 3. In some patients only - cerebellar cortex and deep cerebellar nuclei Atrophy, leading to further reinforcement of the neurological substrate to ataxia, etc. 4. Myocardium in some patients Diffuse loss with scarring. Diagnosis and Differential Diagnosis The characteristic features are: age of onset; insidious start; progressive ataxia with dysarthria; characteristic neurological findings; pes cavus and kyphoscoliosis. Multiple scerosis and subacute combined degeneration differ both in presentation and age of onset. A cerebellar abscess may produce certain similar signs, but pain will usually be present, and progress is much more rapid, quite apart from the results of specialised neurological investigations. There are a number of less common spinocerebellar degenerations to be considered, such a Refsum’s disease, Charcot-Marie-Tooth disease, and the Roussy-Levy syndrome; but these certainly fall into the province of the specialist neurologist.

63. Genetic Testing Zoghbi, HY et al., The spinocerebellar degenerations. In Current Diagnosisin Neurology, ed. E. Feldmann, 87110. 1993. St. Louis Mosby. http://www.athenadiagnostics.com/site/product_search/test_reference_template.asp

64. Rare Disorders Conditions And Diseases Health English Friedreich Ataxia English Health Conditions and Diseases Neurological DisordersSpinal Cord spinocerebellar degenerations Friedreich s Ataxia ? Galactosemia http://www.interactiva.org/Dir/I/English/Health/Conditions_and_Diseases/Rare_Dis

www.interactiva.org English Deutsch Espa±ol ... Rare Disorders Rare Disorders : Health Conditions and Diseases Rare Disorders: Agnosia Aicardi Syndrome Alstrom Syndrome Barth Syndrome ... Wegener's Granulomatosis Aarskog Syndrome: English Health Conditions and Diseases Genetic Disorders Aarskog Syndrome Aase Syndrome: English Health Conditions and Diseases Genetic Disorders Aase Syndrome Abetalipoproteinemia: English Health Conditions and Diseases Neurological Disorders Brain Diseases Metabolic Abetalipoproteinemia Ablepharon-Macrostomia Syndrome: English Health Conditions and Diseases Genetic Disorders Ablepharon-Macrostomia Syndrome Adie Syndrome: English Health Conditions and Diseases Neurological Disorders Autonomic Nervous System Adie Syndrome Adrenoleukodystrophy: English Health Conditions and Diseases Neurological Disorders Demyelinating Diseases Leukodystrophy Adrenoleukodystrophy Alagille Syndrome: English Health Conditions and Diseases Genetic Disorders Alagille Syndrome Alternating Hemiplegia: English Health Conditions and Diseases Neurological Disorders Alternating Hemiplegia Apert Syndrome: English Health Support Groups Conditions and Diseases Facial Differences Apert Syndrome Arthrogryposis: English Health Conditions and Diseases Musculoskeletal Disorders Congenital Anomalies Arthrogryposis Ataxia: English Health Conditions and Diseases Neurological Disorders Ataxia Bardet-Biedl Syndrome: English Health Conditions and Diseases Neurological Disorders Brain Diseases Bardet-Biedl Syndrome Behcet's Syndrome:

65. Understanding Dementia and Pick s disease, subcortical dementia may be caused by Parkinson s disease, Huntington sdisease, Wilson s disease, spinocerebellar degenerations, Lewy body http://www.flonnet.com/fl2006/stories/20030328001108000.htm

Volume 20 - Issue 06, March 15 - 28, 2003 India's National Magazine from the publishers of THE HINDU Home Contents MEDICINE Understanding dementia ASHA KRISHNAKUMAR in Chennai An international workshop in Chennai on dementia underlines the need to create awareness about the problem and improve treatment options and facilities, especially in developing countries like India. N. BALAJI At the 23rd T.S. Srinivasan Endowment Oration on Dementia in Chennai, Venu Srinivasan (left) presenting a memento to Prof. Jeffrey Cummings. Prof. Krishnamoorthy Srinivas (second right) and T.T. Vasu, Chairman, Public Health and Welfare Society look on. IN 1950, the world had 12 people in the working age group to support every person above 65. But by the turn of the millennium, there were hardly nine, and by 2050, it is estimated that there will be barely four persons. With life expectancy at birth increasing rapidly, the world's population of the aged has risen, and with it, their problems. In India, life expectancy at birth has increased by 30 years since Independence, and it is higher for women than men. According to the World Health Organisation (WHO), India's population of those aged over 65, which was 40 million in 1997, is to increase to 108 million by 2025 and 240 million by 2050. This means a several-fold increase in age-related problems such as dementia - a condition characterised by progressively declining memory and intellectual functions. The WHO, which estimates that two out of every three patients with dementia will soon be in developing countries, warns of a virtual dementia epidemic in India and the urgent need to prepare to face it.

66. Eugenio Medea - Scientific Research dysmorphisms; congenital CNS – PNS pathologies; epilepsy; rare syndromes; degenerativediseases – spinocerebellar degenerations; neurosensory disorders. http://www.emedea.it/english_medea/research.html

67. Cerebellar Ataxia ? What Does Everyone Want To Know About Cerebellar Ataxia? Her Ataxia by Patricia Birdsong Hamilton Neural transplantation in cerebellar ataxiaby Lazaros Constantinos Triarhou spinocerebellar degenerations by Tokyo http://health.xq23.com/conditions/part_2/cerebellar_ataxia.html

About cerebellar ataxia : What you and I might want to know. Recommended References and Products. [see index for total category] Books: Cerebellar Degenerations : Clinical Neurobiology (Foundations of Neurology) by Andreas Plaitakis Neural Transplantation in Cerebellar Ataxia by Lazaros Constantinos Triarhou The Hereditary Ataxias and Related Disorders (Clinical Neurology and Neurosurgery Monographs, Volume 6) by A.E. Harding A Balancing ActLiving With Spinal Cerebellar Ataxia by Patricia Birdsong Hamilton Neural transplantation in cerebellar ataxia by Lazaros Constantinos Triarhou Spinocerebellar Degenerations by Tokyo International Symposium on Spinocerebellar Degenerations Serotonin, the Cerebellum, and Ataxia by Paul Trouillas Serendipity Driven Links and Topics: http://www.kyphoto.com/classics/ataxia.html http://www.merck.com/pubs/mmanual/section14/chapter179/179g.htm Latest books on: cerebellar ataxia PUBMED Citations: DVD Videos: Taxi Driver (Special Edition) by Robert De Niro Taxi Driver by Robert De Niro Taxi Driver by Robert De Niro VHS Videos: Taxi Driver (Special Edition) by Robert De Niro Taxi Zum Klo by Frank Ripploh Taxi - Best of Andy Kaufman Vol. 1

68. Šî‘b•a—Šw‡Uu‹`ƒm[ƒg striatonigral dopaminergic system ?. l-dopa. c) spinocerebellar degenerations.1) Olivopontocerebellar atrophy (OPCA) . http://www.showa.gunma-u.ac.jp/~nakazato/pathol/lecture/bp2lect.html

_Œo•a—Šw@ Neuropathology A. Introduction •a•Ï‚Ì•”ˆÊ‚ƏǏó neuron glia (astrocytes, oligodendroglia, microglia, ependymal cells) myelin skull ”]ˆ³˜´iCƒwƒ‹ƒjƒA CSF  “ªÇC×‹ÛEŽîᇂ̊gŽU no lymphatics C. Infection Meningitis leptomeningitis ˆê’UŠ´õ‚ª‹N‚±‚é‚Æ CSF ‚ð‰î‚µ‚Ä”]‘S‘Ì‚Ö a) ‰»”^«‘–Œ‰Š Purulent meningitis, pyogenic m. ×‹Û: E. coli, ƒCƒ“ƒtƒ‹ƒGƒ“ƒU‹Û, ‘–Œ‰Š‹ÛC”x‰Š‹ ‹Û ƒNƒ‚–Œ‚Ì”’‘÷A‚­‚à–Œ‰ºo‚ÌŸøo•¨ micro:@D’†‹ Afibrin @ b) ƒŠƒ“ƒp«‘–Œ‰Š ƒEƒCƒ‹ƒX«C mumps, ECHO, ƒRƒNƒTƒL[ @ c) –«‘–Œ‰Š TB, tuberculous meningitis, ”]’ê•” syphilitic meningitis 2. Cerebral abscess @ a) ŠOCŽèpCskull‚̉ŠÇi’†Ž¨‰ŠA“û“Ë“´‰ŠA•›•@o‰Šj temporal cerebellum frontal base @ b) ŒŒs« nidus .............. necrotic debris, bacteria abscess membrane ... fibrovascular tissue gliosis ............ astrocytes 3. Viral encephalitis ƒEƒCƒ‹ƒX«”]‰Š @ a) Acute viral infection neuron•Ï« - neuronophagia, glial nodules inclusion body - intranuclear intracytoplasmic mononuclear cell infiltration "perivascular cuffing" lymphocytes, plasma cells tropism •a‘ƒ‘I‘𐫠1) Japanese B encephalitis Flavi virus ƒRƒKƒ^ƒAƒJƒCƒGƒJ@@‰Ä`‰H@@ƒƒNƒ`ƒ“‚É‚æ‚范Œ¸ ’W–¾‘ƒ 2) Herpes simplex encephalitis ¬Ž™AÂ”N ’vŽ€—¦70%@‚Ü‚½‚Í’s•ðA‹L‰¯‘rŽ¸ acute necrotizing encephalitis oŒŒA‰óŽ€A“AŠj“à••“ü‘Ì AraC ‚ª’˜Œø b) Slow virus infection 1) Subacute sclerosing panencephalitis (SSPE) measles (defective virus) 2) Progressive multifocal leukoencephalopathy (PML) JC virus 12Î‚Å65%‚ɍR‘Ì—z« c) Prion diseases 1) Creutzfeldt-Jakob disease (CJD) 30 kDa ‚Ì prion’`”’ (proteinous infectious agent)

69. Wauu.DE: Health: Conditions And Diseases: Neurological Disorders: Spinal Cord: S Translate this page Wauu.DE Health Conditions and Diseases Neurological DisordersSpinal Cord spinocerebellar degenerations. http://www.wauu.de/Health/Conditions_and_Diseases/Neurological_Disorders/Spinal_

Home Health Conditions and Diseases Neurological Disorders ... Spinal Cord : Spinocerebellar Degenerations Search DMOZ-Verzeichnis: All Categories Categories Onlye Kategorien: Friedreich Ataxia Links: Marinesco-Sjogren Syndrome Information and a support group for families affected with this syndrome, a rare genetic disorder characterized by ataxia, cataracts, very small stature, and mental retardation. http://www.marinesco-sjogren.org © 1998- 2002 Ein Service von Wauu.de UserNet.DE Linktip: MnogoSearch.ORG Map TopTen TopTen eng Aktuelle Linktips Mozilla 1.7 RC 1 Basteln 0190 Warner Free Rund ums Rad ... Fantasy - Shop Webmaster-Links Webspace Free Subdomain Service Merchandise Fun SUMA - Forum ... Abmahnungen œber Wauu Regeln Webmasterservice Impressum b

70. Wauu.DE: Health: Conditions And Diseases: Neurological Disorders: Spinal Cord: S Wauu.DE Health Conditions and Diseases Neurological DisordersSpinal Cord spinocerebellar degenerations Friedreich Ataxia. http://www.wauu.de/Health/Conditions_and_Diseases/Neurological_Disorders/Spinal_

Home Health Conditions and Diseases Neurological Disorders ... Spinocerebellar Degenerations : Friedreich Ataxia Search DMOZ-Verzeichnis: All Categories Categories Onlye Links: GeneClinics: Friedreich Ataxia An in depth look at this disorder. Includes a summary, diagnosing, molecular genetic testing, clinical description and management. http://www.geneclinics.org/profiles/friedreich/ Living Life with Ataxia An informative site about living a positive life in spite of having a progressive hereditary neurological disease called Friedreich's Ataxia. http://cajunheart.tripod.com/ NINDS Friedreich's Ataxia An information sheet the National Institute of Neurological Disorders and Stroke . http://www.ninds.nih.gov/health_and_medical/disorders/friedreichs_ataxia.htm NORD - Friedreich's Ataxia Offers the synonyms, a general discussion and further resources. © 1998- 2002 Ein Service von Wauu.de UserNet.DE Linktip: MnogoSearch.ORG Map TopTen TopTen eng Aktuelle Linktips Mozilla 1.7 RC 1 Basteln 0190 Warner Free Rund ums Rad ... Fantasy - Shop Webmaster-Links Webspace Free Subdomain Service Merchandise Fun SUMA - Forum ... Abmahnungen œber Wauu Regeln Webmasterservice Impressum b

71. Neurological Disorders Porencephaly (4). Rett Syndrome (10) Personal Pages. Spinal Cord (13)Epidural Abscess,Compression,spinocerebellar degenerations http://www.posterbreak.com/Health/Conditions_and_Diseases/Neurological_Disorders

PosterBreak.com Over 400,000 Posters and Prints to Shop for! Home Contact Search Site Search Web Suggestions: neurological disorder national institute of neurological disorder and stroke disorder institute michigan neurological national institute of neurological disorder ... disorder neurological stroke Links: Health: Conditions and Diseases: Neurological Disorders Home Health Conditions and Diseases : Neurological Disorders Web Results: Daphne, Alabama physician specializes in all types of neurological care and pain management, including epilepsy, sleep disorders, and stroke prevention. Daphne, Alabama physician specializes in all types of neurological care and pain management, including epilepsy, sleep disorders, and stroke prevention. Epilepsy Book Store At WellnessBooks.com Comprehensive source to books on Epilepsy. Includes community boards and chat, book reviews and online ordering. Neurological Disorders Due To An Imbalance? Do you have a neurological disorder? This may be due to an imbalance. Check the balance of your system and get natural recommendations for restoring balance. Subcategories: Alternating Hemiplegia Alzheimer's Directories Personal Pages ... Spinal Cord Injury Neurological Disorders- More Web Results Below Brain.com

72. Untitled Document ATAXIA; spinocerebellar degenerations. Referencias bibliográficas. http://www.bvs.sld.cu/revistas/ibi/vol22_3_03/ibi09303.htm

Anterior Siguiente Rev Cubana Invest Biomed 2003:22(3):199-203 Formato PDF Centro de Investigaciones Biomédicas "Victoria de Girón" Resumen DeCS: ESTRES OXIDATIVO; TRASTORNOS HEREDODEGENERATIVOS DEL SISTEMA NERVIOSO; ATAXIA; DEGENERACIONES ESPINOCEREBELOSAS. Desarrollo Ataxia de Friedreich Estudios recientes indican que la proteína ATM aparece cuando ocurren roturas en la doble cadena del ADN durante la meiosis o la mitosis, o como consecuencia del daño causado por los radicales libres. También se ha descrito que el producto del gen ATM fosforila directamente a p53, factor de transcripción que se activa en situaciones de estrés celular, y que además interactúa con otras moléculas que participan en la reparación, así como en la señalización celular como ATR, chk-1, chk-2, RPA, BRCA1, BRCA2, NF kappab, entre otros; por lo que se conoce como la "quinasa jerárquica". Ataxia con deficiencia de vitamina E a -tocoferol trasferasa ( a -TTP).

73. Baylor Neurology Case Of The Month Most of the spinocerebellar degenerations have relatively late onsets (beyond theage of puberty), and most demonstrate some degree of cerebellar, pyramidal http://www.bcm.tmc.edu/neurol/challeng/pat25/summary.html

Patient #25 Summary and Discussion Diagnosis: Friedreich's Ataxia Patient #25 presented with progressive gait and limb ataxia, mild distal symmetrical sensory loss, dimished deep tendon reflexes, weakness of the gluteal muscles, and bilateral extensor plantar responses. These findings indicate cerebellar, peripheral nerve (or dorsal root ganglion), and corticospinal involvement and imply a multisystem degenerative disease. There was no evidence of autonomic dysfunction, and eye movements were left unaffected. Extrapyramidal involvement was not present arguing against one of the Multi-System Atrophies (MSAs), such as olivopontocerebellar atrophy (OPCA). The most salient feature in this case is the patient's marked progressive ataxia. Ataxia may be due either to cerebellar or proprioceptive dysfunction, though it is rarely difficult to distinguish the two. However, when both are present, diagnostic difficulties arise. The findings in this case - gait and limb ataxia, titubation, loss of check response, and dysdiadochokinesia - point to an abnormality in the cerebellar system. This patient also had evidence of a peripheral neuropathy with involvement of the posterior columns evidenced by decreased position and vibratory sense. The degree of proprioceptive abnormality was not sufficient to explain the marked gait disturbance, however. While this patient exhibited dysfunction in both the cerebellar and proprioceptive systems, the cerebellar involvement was most impressive. The primary defect, therefore lies somewhere in the connections to, from, or within the cerebellum.

74. Nervous System Disease --  Encyclopædia Britannica Acquired diseases of muscle. The spinal cord spinocerebellar degenerations;Inflammation; Trauma; Tumours; Subacute combined degeneration; http://www.britannica.com/eb/article?eu=119952&tocid=75751&query=tinnitus&ct=

75. Nervous System Disease --  Encyclopædia Britannica spinocerebellar degenerations are a group of inherited disorders characterizedby atrophy of the central nervous system and of peripheral nerves. http://www.britannica.com/eb/article?eu=119952&tocid=75758&query=scoliosis&ct=

76. Summary The patients were classified according to the criteria made by the Research Committeeon spinocerebellar degenerations of The Japanese Ministry of Welfare and http://www.m.chiba-u.ac.jp/med-journal/71/71-3/713ef.html

77. Ramsay Hunt's Syndrome I (www.whonamedit.com) rare. It has been classified under the spinocerebellar degenerations.Some of the cases are due to mitochondrial abnormalities. http://www.whonamedit.com/synd.cfm/2245.html

Home List categories Eponyms A-Z Biographies by country ... Contact Whonamedit.com does not give medical advice. This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor. Ramsay Hunt's syndrome I Also known as: Hunt’s ataxia Hunt’s syndrome I Ramsay Hunt’s syndrome II Synonyms: Dentate cerebellar ataxia; dentatorubral atrophy, dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dyssynergia cerebellaris progressiva sive myoclonica, primary dentatum atrophy. Associated persons: James Ramsay Hunt Description: A rare form of progressive cerebellar dyssynergia mainly characterised by intention tremor and often associated with convulsions and myoclonic epileptic jerks. Tremor usually begins locally in one extremity and spreads gradually, eventually involving the entire voluntary motor system. Legs are disturbed less often than arms Other features include unsteady gait, errors in estimating the range, direction, and force of voluntary movements, muscular hypotonia, asthenia, and adiadochokinesia are associated. The cause is uncertain, it may be due to a degeneration of the olivodentatorubral system. Onset usually in early adulthood, the average is 30 years. Mental deterioration occurs but is rare. It has been classified under the spinocerebellar degenerations. Some of the cases are due to mitochondrial abnormalities. Autosomal dominant inheritance with reduced penetrance suggested.

78. Neurological Disorders Porencephaly(4). Rett Syndrome(10) Personal Pages. Spinal Cord(13) EpiduralAbscess, Compression, spinocerebellar degenerations, Tabes Dorsalis. http://www.jaspergifts.com/Health/Conditions_and_Diseases/Neurological_Disorders

Neurological Disorders JasperGifts.com Shopping? It's all here! Shopping Contact Top Trade ... Webmasters Search Aromatherapy Baby Bath and Body Bed and Bath ... disorder neurological stroke Links: Health: Conditions and Diseases: Neurological Disorders Home Health Conditions and Diseases : Neurological Disorders Daphne, Alabama physician specializes in all types of neurological care and pain management, including epilepsy, sleep disorders, and stroke prevention. Daphne, Alabama physician specializes in all types of neurological care and pain management, including epilepsy, sleep disorders, and stroke prevention. Epilepsy Book Store At WellnessBooks.com Comprehensive source to books on Epilepsy. Includes community boards and chat, book reviews and online ordering. Neurological Disorders Due To An Imbalance? Do you have a neurological disorder? This may be due to an imbalance. Check the balance of your system and get natural recommendations for restoring balance. Subcategories: Alternating Hemiplegia Alzheimer's Directories Personal Pages ... Spinal Cord Injury Neurological Disorders- More Web Results Below Add Your Site or modify Brain.com

79. DiseaseSeek.com Spinocerebellar_Degenerations Conditions and Diseases Neurological Disorders Spinal Cord SpinocerebellarDegenerations Found 0 sites about spinocerebellar degenerations. http://www.diseaseseek.com/categories/Health__Conditions_and_Diseases__Neurologi

@import url(http://www.animationseek.com/style.css); Search Directory Forum Conditions and Diseases Neurological Disorders Spinal Cord Spinocerebellar Degenerations - Found sites about Spinocerebellar Degenerations Add / Update Url Become an Editor Link to Us Sitemap ... DiseaseSeek.com - Medical Conditions and Disease Search Engine - Seeking Sites about Conditions, Allergies,Blood Disorders,Cancer, Cardiovascular Disorders,Communication Disorders, Congenital Anomalies,Dental Disorders,Digestive Disorders,Ear, Nose and Throat, Endocrine. MedicineSeek.com AstronomySeek.com BiologySeek.com ChemistrySeek.com ... TechnologySeek.net

80. Myoclonus spinocerebellar degenerations, basal ganglia degenerations, mitochondrial disorders),and other dementing illnesses (JakobCruetzfeldt disease, Alzheimers http://www.cmdg.org/Movement_/myoclonus/myoclonus.htm

The Canadian Movement Disorder Group MYOCLONUS Definition: A sudden "Shock" like muscular movement Myoclonus can be Classified By: 1) The Electrophysiologic Phenomenon (certain electrical characteristics) Negative Myoclonus (asterixis) a sudden  relaxation of a group of muscles. 2) Classification by the Anatomical Origin Electical Discharge From the surface of the brain ( Cortical Myoclonus ).............Epileptic From deeper within the brain ( Subcortical Myoclonus From an electrical impulse within the spinal cord ( Spinal or Segmental Myoclonus From an electrical impulse within a peripheral nerve ( Peripheral Myoclonus eg. hemifacial spasm) 3) Classification byBody Parts Involved Focal If only one body part is affected myoclonus is called "focal" . (eg. myoclonic jerks of 1/2 the face ( "hemifacial spasm" ), or when the eyes are affected "Opsoclonus" Segmental If only one segment of the body is affected myoclonus is called "segmental"

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