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Soonchunhyang Medical Science 18(2):122-124, December 2012 pISSN: 2233-4289 I eISSN: 2233-4297 CASE REPORT A Case of Isolated Thyroid Stimilating Hormone Deficiency Hyun Wook Cho, Yeo Joo Kim, Sang Jin Kim Division of Endocrinology, Soonchunhyang University Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Korea Isolated thyroid stimulating hormone (TSH) deficiency is very rare. Only a few patients are reported in the world. A 50-year-old wo man was admitted to Soonchunhyang University Cheonan Hospital because of a distal radius fracture. She was diagnosed with hypothyroidism incidentally. Serum free T4 and TSH were low. After administration of exogenous TSH releasing hormone, no TSH response was noted. The response of prolactin and other pituitary hormones were appropriate after stimulation. Pituitary magnetic resonance image did not reveal any abnormalities. The diagnosis of isolated TSH deficiency was established for this patient. Keywords: Hypothyroidism; Thyrotropin; Deficiency INTRODUCTION edwithisolatedTSHdeficiencywithareviewofthemedicalliterature. Hypothyroidismisacommondisease.Theprevalenceofhypothyroidismin2009forKoreaincluded248,387people.Hypothy- CASE REPORT roidismcanbeclassifiedasprimaryhypothyroidism,transient causeandcentralone.Secondaryhypothyroidismreferstoahy- A50-year-oldwomanwithahistoryofhypothyroidismfor6 pothyroidstateassociatedwiththyroidstimulatinghormone(TSH) monthspresentedtoSoonchunhyangUniversityCheonanHospi- deficiency.WiththeavailabilityofTSHreleasinghormone(TRH) talforpainintherightwrist.Radiologicimagingconfirmedadis- assay,itisnowpossibletoseparatepituitaryoriginfromhypotha- talradiusfracturebyslipdown.Shewasdiagnosedwithhypothy- lamichypothyroidism. roidismduringaroutinehealthscreenatanotherfacility6months Hypothyroidismismostlycausedbyprimaryhypothyroidism. priortopresentation.However,despitebeingrecommendedto Hashimoto’sthyroiditisisthemostcommoncauseofprimaryhy- takethyroidhormone,shedidnottakeanyofthemedication.She pothyroidism.Secondaryhypothyroidismisnotcommonandis hadnopasthistoryofcardiacorrenaldisease,andtherewasno usuallydiagnosedinthecontextofotheranteriorpituitaryhor- historyofsignificantalcoholintake,smokingorprevioussurgery. monedeficiencies,ifitsoriginisfromthepituitarygland.Pitu- Herfamilyhistorywasalsounremarkable.Uponhistorytaking itarydiseaseswithmultipleanteriorpituitaryhormonedeficien- andphysicalexam,shedidnotexhibitanymanifestationforhy- ciesusuallyoccurinapredictablesequence,withgrowthhormone pothyroidism. (GH)andgonadotropindeficiencyprecedingthatofTSHandadrenocorticotropin. Onadmissionherbloodpressurewas120/80mmHgwitha pulserateof80beats/min.Physicalexaminationwasunremark- However,isolatedTSHdeficiencyisveryrareworldwide.Itwas ableanddisplayednospecificabnormalitiesbesidesherradius firstreportedin1953byShuman[1].Sincethenonlyafewsporad- fracture.Laboratorydatashowednoabnormalfindingsexceptin iccaseshavebeenreported,andtherehavebeenonlytworeported herthyroidfunctions:serumwhitebloodcellcountwas4,350/μL; casesinKorea[2].WepresentthecaseofaKoreanfemalediagnos- hemoglobin12.8g/dL;bloodureanitrogen17.9mg/dL;creatinine Correspondence to: Yeo Joo Kim Division of Endocrinology, Soonchunhyang University Cheonan Hospital, Soonchunhyang University College of Medicine, 31 Suncheonhyang 6-gil, Dongnam-gu, Cheonan 330-930, Korea Tel: +82-41-570-3685, Fax: +82-41-574-5762, E-mail: [email protected] Received: Jun. 22, 2012 / Accepted after revision: Oct. 9, 2012 122 http://jsms.sch.ac.kr © 2012 Soonchunhyang Medical Research Institute This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/). Secondary Hypothyroidism, TSH Deficiency • Cho HW et al. Table 1. Results of combined pituitary secretion test Reference value 0 min 15 min 30 min 60 min 90 min 120 min Glucose (mg/dL) GH (mIU/L) FSH (mIU/mL) LH (mIU/mL) TSH (µIU/mL) ACTH (pg/dL) Cortisol (µg/dL) Prolactin (ng/mL) 70-130 90 53 67 31 64 114 0.06-5.0 8.34 4.41 16.47 49.5 52.2 29 25-134 38.05 44.06 49.88 56.59 52.24 53.28 7.7-58 21.09 38.76 51.78 60.06 51.49 47.75 0.27-4.2 0.532 1.76 2.29 1.76 1.16 0.951 5-60 24 36 178 366 266 93 6.1-19 3.64 7.75 11.17 26.07 27.98 26.06 4.6-21 7.26 69.87 76.04 106.5 58.15 33.18 ACTH, adrenocorticotropic hormone; FSH, follicle stimulating hormone; GH, growth hormone; LH, luteinizing hormone. ranges:TSH0.046µIU/mL;freeT40.946ng/dL;T31.13ng/mL. DISCUSSION Mostcommoncausesofprimaryhypothyroidismareprimary, arisingfromautoimmunethyroiditisandvariousotheriatrogenic causes.Centralhypothyroidism,whichisfromeitherapituitary orahypothalamicorigin,isclassifiedassecondaryhypothyroidism,whichisnotamajorcauseofhypothyroidism. PituitaryhypothyroidismischaracterizedbylowbasalTSH A B Fig. 1. (A, B) Pituitary magnetic resonance imaging did not show any abnormalities in Sella turcica. levelsinthesettingoflowfreethyroidhormone.Patientswithhypothyroidismofhypothalamicorigin,ontheotherhand,mayexhibitnormalorevenslightlyelevatedTSHlevels.TheTSHproducedinthelattercircumstanceappearstohavereducedbiologic 0.7mg/dL;serumsodiumlevel145mEq/L;serumpotassiumlevel activityduetoalteredglycosylation[3].HyposecretionofTSHin 4.1mEq/L;aspartateaminotransferase33IU/L;alanineamino- pituitaryhypothyroidismisascribedtoareducedmassoffunc- transferase23IU/L;serumglucose103mg/dL.Herthyroidfunc- tioningthyrotrophsasaconsequenceofvariouslesionsthatcan tiontestshowedhypothyroidismbasedonlowTSH(0.066µIU/ includemechanicalcompressionbytumor,destructionbyvascu- mL;normalrange,0.27to4.2);lowfreeT4(0.799ng/dL;normal lar,inflammatoryorphysicalinjuries,alpasia,orhypoplasia.In range,0.93to1.7),andnormalT3(1.03ng/mL;normalrange,0.8 addition,itmayalsobeidiopathic,whenthereisnoevidenceof to2.0).Antibodytothyroglobulinwas520IU/mL(normalrange, injurytothepituitaryorpresenceofanyneoplasticlesioninthe 0to115IU/mL)andtheanti-thyroidperoxidaseantibodywas600 brain.IdiopathicTSHdeficiencytakesplaceswithcorrelationto IU/mL(normalrange,0to34IU/mL).Thyroidultrasonography GHdeficiency[4],butsecretionofotherpituitaryhormonesmay showednoabnormailities.Theseresultsindicatedherhypothy- alsobeimpaired[5].Althoughmorecommoninadults,isolated roidismtobeofsecondaryorigin.Theresultsofcombinedanteri- TSHdeficiencyalsooccursinchildrenandmayresultinasecond- orpituitarysecretiontestareshowninTable1.Afteradministra- aryimpairmentofGHsecretion,includingprimarydeficiencyof tionofexogenousTRH,noTSHresponsewaselicited.However, TSHandGH[6]. herprolactinandotherpituitaryhormoneresponsesafterstimu- IsolatedTSHdeficiencycanoccurbutisveryrare.Severalex- lationwerenormal.ChestX-raywaswithinnormallimitsandher planationsforidiopathicisolatedTSHdeficiencyarepossible.Pro- electrocardiogramshowedanormalsinusrhythm.Pituitarymag- videdthatthethyrotrophsarepresentandmorphologicallyintact, neticresonanceimaging(MRI)didnotshowanyabnormalities theabnormalitycouldresideintheTRHreceptororatsomesub- (Fig.1).Thepatientwastreatedwithlevothyroxine50μgperday. sequentstepinthetransmissionofthehypothalamicmessage,in After2monthsoftreatment,thyroidfunctionreturnedtonormal theprocessofTSHsynthesis,orTSHrelease.Anyabnormalitiesin Soonchunhyang Medical Science 18(2):122-124 http://jsms.sch.ac.kr 123 Cho HW, et al. • Secondary Hypothyroidism, TSH Deficiency theTRHreceptordonotinvolveprolactin-secretingcellsbecause roidism.However,ifthepatienthasanisolatedTSHdeficiency,it theprolactinresponsetoTRHisnormalinthesepatients[7]. mustnotbeoverlookedthatthelesionresponsibleforthedisease InheritedisolatedTSHdeficiencyisanautosomalrecessivedis- mayleadtootherhormonaldeficiencieslateron.Thus,whenapa- easethatresultsincongenitalhypothyroidismandhasbeenre- tienthasbeendiagnosedwithisolatedTSHdeficiency,thephysi- portedafewtimes.Itisrelatedtoeitherasingle-basesubstitution cianshouldbealertforanysubsequentchangesthatmayrequire ortoanonsensemutationintheTSH-subunitgene,anddefectsin additionalevaluationofotherhormonelevelsandtherespective pituitaryspecifictranscriptionfactorhavebeenalsodescribed[8]. therapyforeachhormoneabnormalitiesasindicated[6].Were- ThediagnosticcriteriaforisolatedTSHdeficiencyinclude1)a portthiscasetomakenoteofthefactthatboththediagnosisand lowlevelofthyroidhormoneswithlowTSHlevelintheserum, follow-upcareofisolatedTSHdeficiencymustbemeticulously withnormalorhighserumTRHlevels,2)theabsentresponseof done. serumTSHtoexogenousTRHadministration,butwithanormal serumprolactinlevel,and3)normalsecretionsofotheranterior pituitaryhormones.ThelaboratorydataacquiredfromourpatientsiscompatiblewithisolatedTSHdeficiency.Thecauseofher isolatedTSHdeficiencywasnotfoundinherpituitaryMRI.Ageneticanalysiswasnotconductedduetoherrefusal. Themainclinicalsymptomsofhypothyroidismarefatigue, weakness,dryskin,feelingcold,hairloss,constipation,dyspnea, hoarseness,andparesthesia.Thetypicalclinicalfeaturesincludea puffyfacewithedematouseyelids.However,adultpatientswith isolatedTSHdeficiencyaregenerallypresentedwithabsentormild symptomsofhypothyroidism[9].Inoutpatient’scase,shewasdiagnosedincidentallywithhypothyroidismalthoughnosymptoms oftheconditionwereshown. TreatmentofTSHdeficiencyisadailyreplacementwithlevothyroxine.Itshouldbeinitiatedafteradequateadrenalfunction hasbeenestablished.DoseadjustmentisbasedonthyroidhormonelevelsandclinicalparametersratherthantheTSHlevel[10]. Sincethetypicalsymptomswithhypothyroidismareusually lacking,itisclinicallyimportanttomakethediagnosisofisolated TSHdeficiency.Administeringthyroidhormone,oncethediag- REFERENCES 1. Shuman CR. Hypothyroidism due to thyrotropin deficiency without other manifestations of hypopituitarism. J Clin Endocrinol Metab 1953; 13:795-800. 2. Choi SJ, Kim WS, Kim SH, Kim H, Park SM, Lee WK, et al. A case of isolated TSH deficiency. Endocrinol Metab 1999;14:392-5. 3. Devdhar M, Ousman YH, Burman KD. Hypothyroidism. Endocrinol Metab Clin North Am 2007;36:595-615. 4. Gharib H, Abboud CF. Primary idiopathic hypothalamic hypothyroidism: report of four cases. Am J Med 1987;83:171-4. 5. Streja D, Corenbium B, Ezrin C. Hypothalamic hypopituitarism presenting as galactorrhea-amenorrhea. JAMA 1978;239:1783-5. 6. Samuels MH, Ridgway EC. Central hypothyroidism. Endocrinol Metab Clin North Am 1992;21:903-19. 7. Beck-Peccoz P, Amr S, Menezes-Ferreira MM, Faglia G, Weintraub BD. Decreased receptor binding of biologically inactive thyrotropin in central hypothyroidism: effect of treatment with thyrotropin-releasing hormone. N Engl J Med 1985;312:1085-90. 8. Hayashizaki Y, Hiraoka Y, Tatsumi K, Hashimoto T, Furuyama J, Miyai K, et al. Deoxyribonucleic acid analyses of five families with familial inherited thyroid stimulating hormone deficiency. J Clin Endocrinol Metab 1990; 71:792-6. 9. Lohrenz FN, Fernandez R, Doe RP. Isolated thyrotropin deficiency: review and report of three cases. Ann Intern Med 1964;60:990-8. 10. Lania A, Persani L, Beck-Peccoz P. Central hypothyroidism. Pituitary 2008;11:181-6. nosishasbeenmade,istheonlytherapyfitforprimaryhypothy- 124 http://jsms.sch.ac.kr Soonchunhyang Medical Science 18(2):122-124