Sickle Cell Disease: Report of a Case With Cerebral Manifestations in the Absence of Anemia

Case Reports1 November 1948SICKLE CELL DISEASE: REPORT OF A CASE WITH CEREBRAL MANIFESTATIONS IN THE ABSENCE OF ANEMIARAYMOND K. THOMPSON, M.D., JOHN A. WAGNER, M.D., CHRISTIE M. MACLEOD, M.D.RAYMOND K. THOMPSON, M.D., JOHN A. WAGNER, M.D., CHRISTIE M. MACLEOD, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-29-5-921 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptSickle cell disease was originally described by Herrick1in 1910, as anemia with sickle-shaped erythrocytes. During a crisis of sickling these characteristic cells become wedged in capillaries, often producing thrombosis. In the past 20 years increasing attention has centered about the histopathology of sickle cell disease, particularly with reference to the vascular thromboses so frequently noted. This vascular occlusive phenomenon results in a protean symptomatology dependent upon the vessels occluded. Although the cerebral manifestations of the disease were undoubtedly previously noted, the first report was that of Sydenstricker, Mulherin, and Houseal2in 1923. This was followed by isolated case reports.3-22...Bibliography1. HERRICK JB: Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia, Trans. Assoc. Am. Phys., 1910, xxv, 553-561. Google Scholar2. SYDENSTRICKERMULHERINHOUSEAL VPWARW: Sickle cell anemia; report of two cases in children with necropsy in one case, Am. Jr. Dis. Child., 1923, xxvi, 132-154. CrossrefGoogle Scholar3. GRAHAM GS: A case of sickle cell anemia with necropsy, Arch. Int. Med., 1924, xxxiv, 778-800. CrossrefGoogle Scholar4. HAMILTON JF: A case of sickle cell anemia, Memphis Med. Jr., 1925, ii, 253-257. Google Scholar5. BELLKOTTEMITCHELL AJRHAG: Sickle cell anemia; reports of two cases in young children in which splenectomy was performed, Am. Jr. Dis. Child., 1927, xxxiv, 923-933. CrossrefGoogle Scholar6. COOK WC: A case of sickle cell anemia with associated subarachnoid hemorrhage, Jr. Med., 1930, xi, 541. Google Scholar7. LIEVYSCHNABLE FETG: Abdominal crises in sickle cell anemia, Am. Jr. Med. Sci., 1932, clxxxiii, 381-391. CrossrefGoogle Scholar8. ANDERSONWARE WWRL: Sickle cell anemia, Am. Jr. Dis. Child., 1932, xliv, 1055-1070. Google Scholar9. CORRIGANSCHILLER JCIW: Sickle cell anemia; report of 8 cases, 1 with necropsy, New England Jr. Med., 1934, ccx, 410-417. CrossrefGoogle Scholar10. ARENA JM: Vascular accident and hemiplegia in a patient with sickle cell anemia, Am. Jr. Dis. Child., 1935, xlix, 722-723. Google Scholar11. PENBERTHYCOOLEY GCTB: Results of splenectomy in childhood, Ann. Surg., 1935, cii, 645-655. CrossrefGoogle Scholar12. RYERSONTERPLAN CSKL: Sickle cell anemia; two unusual cases with autopsy, Folia haemat., 1935, liii, 353-369. Google Scholar13. BAIRD JA: Sickle cell anemia; report of case with multiple infarctions and necropsy, Med. Bull. Vet. Admin., 1934, xi, 169-171. Google Scholar14. YATERHAUSMANN WMGH: Sickle cell anemia; new cause for cor pulmonale; report of 2 cases with numerous disseminated occlusions of small pulmonary arteries, Am. Jr. Med. Sci., 1936, cxci, 474-484. CrossrefGoogle Scholar15. KAMPMEIER RH: Sickle cell anemia as a cause of cerebral vascular disease, Arch. Neurol. and Psychiat., 1936, xxxvi, 1323-1329. CrossrefGoogle Scholar16. PENNA DE AZEVEDO A: Sombre o diagnostico histologico da anamia de panocytica, Men. Inst. Oswaldo, Cruz., 1937, xxxii, 517-520. CrossrefGoogle Scholar17. BOSSELMANKRAINES BSH: Mental changes including aphasia in a patient with sickle cell anemia, Am. Jr. Psychiat., 1937, xciv, 709-712. CrossrefGoogle Scholar18. FORD FR: Sickle cell anemia, in Diseases of the Nervous System in Infancy, Childhood, and Adolescence, 1937, Chas. C. Thomas, Springfield, Ill., p. 642. Google Scholar19. ARENA JM: Cerebral vascular lesions accompanying sickle cell anemia, Jr. Pediat., 1939, xiv, 745-751. CrossrefGoogle Scholar20. BRIDGERS WH: Cerebral vascular disease accompanying sickle cell anemia, Am. Jr. Path., 1939, xv, 353-362. Google Scholar21. SKOGG AL: Cerebral complications in sickle cell anemia, South. Med. Jr., 1940, xxxiii, 714-721. CrossrefGoogle Scholar22. JOSEY AI: Sickle cell anemia with cerebral thrombosis, South. Med. Jr., 1939, xxxii, 915-917. CrossrefGoogle Scholar23. HUGHESDIGGSGILLESPIE JGLWCE: The involvement of the nervous system in sickle cell anemia, Jr. Pediat., 1940, xvii, 166-184. CrossrefGoogle Scholar24. YOUNGEMCINTOSH WAEF: Case report, sickle cell anemia with hemiplegia, Jr. Nat. Med. Assoc., 1941, xxxiii, 130-132. Google Scholar25. WALKERMURPHEY DWJP: Sickle cell anemia complicated by acute rheumatic heart disease and massive cerebral hemorrhage, Jr. Pediat., 1941, xix, 28-37. CrossrefGoogle Scholar26. WADESTEVENSON LJLD: Necrosis of bone marrow with fat embolism in sickle cell anemia, Am. Jr. Path., 1941, xvii, 47-54. Google Scholar27. CONNELL JH: Cerebral necrosis in sickle cell disease, Jr. Am. Med. Assoc., 1942, cxviii, 895. Google Scholar28. WERTHAMMITCHELLANGRIST FNA: Brain in sickle cell anemia, Arch. Neurol. and Psychiat., 1942, lvii, 752-767. CrossrefGoogle Scholar29. BAUER J: Sickle cell disease; pathogenic, clinical and therapeutic considerations, Arch. Surg., 1940, xli, 1344-1362. CrossrefGoogle Scholar30. BAUERFISHER JLJ: Sickle cell disease with special regard to its nonanemic variety, Arch. Surg., 1943, xlvii, 553-563. CrossrefGoogle Scholar31. MURPHYSHAPIRO RCS: The pathology of sickle cell disease, Ann. Int. Med., 1945, xxiii, 376-397. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Middletown, Connecticut*Received for publication September 5, 1947.†Resident in Neurosurgery, University of Maryland Hospital, Baltimore, Maryland.‡Assistant Professor, Neurosurgery and Neuropathology, University of Maryland Medical School.§Department of Laboratories, Middlesex Hospital, Middletown, Connecticut. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byProspective Brain Imaging Evaluation of Children with Sickle Cell Trait: Initial ObservationsSuperior sagittal sinus thrombosis with infarction in sickle cell trait.Cerebral infarction in sickle cell traitSickle Cell Trait and Multiple Cerebral InfarctionsThe morbidity of sickle cell traitThe Electroencephalogram in Neurological Complications of Sickle Cell Anemia (SS-Hemoglobinopathy)Neurological Manifestations in Sickle-Cell Disease With a Review of the Literature and Emphasis on the Prevalence of HemiplegiaBARRY A. PORTNOY, M.D., JOHN C. HERION, M.D.Anterior and Posterior Hypopituitarism Associated with Sickle Cell TraitROBERT A. PASTURE, JAMES W. ANDERSON, ROBERT H. HERMANNiere bei Tumoren, Blutkrankheiten und ParaproteinämienSickle Cell Trait and Superior Longitudinal Sinus ThrombosisERIC A. SCHENK, M.D.Fatal Intravascular Sickling in a Patient with Sickle-Cell TraitDas Zentralnervensystem bei Krankheiten des BlutesSICKLEMIA*†NORMAN ENDE, M.D., PHILIP PIZZOLATO, M.D., JOSEPH ZISKIND, M.D., F.A.C.P.The minute blood vessels in diseaseTHE THERAPEUTIC RESPONSE OF TUBERCULOUS NEGROES WITH THE SICKLE CELL TRAIT*WILLIAM WEISS, M.D.Sickle-cell DiseaseConstitutional Pathology of ErythropoiesisStudies in sickle-cell anemiaOCCURRENCE OF SYMPTOMS OF SICKLE CELL DISEASE IN THE ABSENCE OF PERSISTENT ANEMIA*THOMAS W. GREEN, M.D., C. LOCKARD CONLEY, M.D.Sickle-cell disease 1 November 1948Volume 29, Issue 5Page: 921-928KeywordsAnemiaCapillariesCellsHistopathologyNeuropathologyNeurosurgerySickle cell diseaseThrombosis ePublished: 1 December 2008 Issue Published: 1 November 1948 PDF downloadLoading ...