An 11-year-old girl presented with sudden sensory disturbance and left-sided muscle weakness. MRI revealed ischaemic change in the right lateral thalamus and the right internal capsule. During sonographic work-up of the cervical arteries, inflammation of the thyroid gland was noted. The results of the thyroid function tests and antibody titers confirmed Hashimoto thyroidits. Under high-dose corticosteroids, the girl had a full neurological recover

Stranzinger, Enno

Zimmermann, Petra

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CASE REPORTSteroid-responsive encephalopathy associatedwith Hashimoto thyroiditisPetra Zimmermann & Enno StranzingerReceived: 17 May 2011 /Revised: 19 September 2011 /Accepted: 24 September 2011 /Published online: 30 December 2011# Springer-Verlag 2011Abstract An 11-year-old girl presented with sudden sensorydisturbance and left-sided muscle weakness. MRI revealedischaemic change in the right lateral thalamus and the rightinternal capsule. During sonographic work-up of the cervicalarteries, inflammation of the thyroid gland was noted. Theresults of the thyroid function tests and antibody titers con-firmed Hashimoto thyroidits. Under high-dose corticoste-roids, the girl had a full neurological recovery.Keywords Hashimoto thyroiditis . Cerebrovascular event .Enchephalopathy . ChildIntroductionHashimoto encephalopathy is a steroid-responsive enceph-alopathy associated with elevated concentrations of antithy-roid antibodies. Patients with the condition are usuallyeuthyroid or mildly hypothyroid. The pathogensis of Hashi-moto encephalopathy is not fully understood, but evidencefor autoimmune and vasculitic mechanisms exist [1]. Thedisease usually responds well to corticosteroids. There aretwo subtypes. The vasculitic type is characterised by acutestroke-like episodes with focal neurological deficits,myoclonus and seizures. The other subtype is diffuse,progressive and associated with insidious onset and progres-sive impairment of mental status, such as confusion, hallu-cinations, mood disturbances and psychosis. We report a girlwho presented with an acute stroke implying that she suf-fered from the vasculitic type of Hashimoto encephalopathy.Case reportA previously healthy 11-year-old girl presented to the emer-gency room with sudden onset of frontal headache, sensorydisturbance and left-sided weakness. There was no historyof nausea or vomiting. She had left facial nerve palsy. Therewas mild hypoaesthesia, hypoalgesia, acrognosis and re-duced muscle strength in her left arm and leg. Diffusion-weighted MRI showed a 10-mm hyperintense area withmatching low apparent diffusion coefficients (suggestingrestricted diffusion) in the right thalamus and internal cap-sule (Fig. 1). This was consistent with an acute stroke. Thegirl was admitted to hospital and treatment with antiplateletdrugs was started.Haematological, rheumatological, renal and liver bio-chemical tests were normal. Cerebrospinal fluid, serologicaltests for borreliosis and ECG were all unremarkable. Ultra-sound (US) was preformed to rule out abnormalities of thearteries in the neck. These were normal, but there wassymmetrical diffuse enlargement of the thyroid gland witha hypoechoic, heterogeneous mirconodular echo pattern. Oncolour Doppler, a diffuse increase in thyroid parenchymal-vascularity was noted (Fig. 2). These findings suggestedHashimoto thyroiditis.Thyroid function tests showed slightly elevated thyroid-stimulating hormone of 5.71 mU/l (normal range, 0.35–5)with normal levels of free-T4 and free-T3. A markedlyelevated antithyroperoxidase antibody titer was observed atP. Zimmermann (*)Department of Paediatrics,University Children’s Hospital, Inselspital,Freiburgstrasse, Bern 3010, Switzerlande-mail: petra.zimmermann@insel.chE. StranzingerInstitute of Radiology – Paediatric Radiology,University Hospital Bern,Bern, SwitzerlandPediatr Radiol (2012) 42:891–893DOI 10.1007/s00247-011-2309-72,313 IU/mL (normal range, < 33 IU/ml). The antithyroglo-bulin antibody level was normal at 46 IU/mL (normal range,< 100 IU/mL). These test results together with the USfindings led to the diagnosis of a Hashimoto thyreoiditis.There was no clinical goitre.Intravenous therapy with high-dose corticosteroid wasinitiated. Subsequently all neurological symptoms and find-ings resolved. After 5 days, she was discharged with anti-platelet drugs and oral corticosteroids to be taken for3 months. Follow-up MRI after 10 weeks showed a smallhyperintense lesion in the right thalamus on T2-weightedimaging, and this was interpreted as gliosis. There was nofurther abnormality.DiscussionThe differential diagnosis of paediatric strokes is muchwider than in adults and includes arteriopathy, cardiac dis-orders, infections and other acute and chronic systemicconditions. Therefore, cerebral MRI, chest radiograph andneck vessel US are part of the investigation.Corticosteroid-responsive encephalopathy associatedwith Hashimoto thyroiditis has chiefly been reported inadults, but is a well-recognised differential diagnosis ofencephalopathy in children [2] in whom it is often over-looked. As our case illustrates, this condition is treatable,and therefore clinical examination of the thyroid glandFig. 1 Cranial MRI in an 11-year-old girl. a Axial T2-weighted imagesshow a 10-mm hyperintense blurred lesion of the right thalamus andinternal capsule (arrow). b Diffusion-weighted image demonstrateshyperintensity related to restricted diffusion in the same area (arrow). cApparent diffusion coefficient map shows the corresponding low valuesin the area (arrow)Fig. 2 Transverse sonogram ofthe thyroid gland. a B-modeultrasound (US) reveals hetero-geneous enlargement of thegland with small focal hypoe-choic nodules (arrow) compati-ble with lymphocytic infiltration.b Increased vascularity of thegland is seen on colour Doppler892 Pediatr Radiol (2012) 42:891–893needs to be performed in children with unclear encephalop-athy. At neck vessel US, it is advisable to include thethyroid. Patients with chronic Hashimoto thyroiditis areusually hypothyroid and show elevated levels of thyroid-stimulating hormone. This has a hypertrophic effect on thethyroid gland and enlargement can be seen on US. ColourDoppler US often reveals hypervascularity within the gland[3], as in our report.Brain MRI in children with Hashimoto encephalopathymay show focal hyperintensity or volume depletion of thenucleus accumbens, frontal white matter changes, cerebellaratrophy and hippocampal and periventricular lesions.SPECT and MR perfusion imaging may show focal orbilateral hypoperfusion [4]. As in our report, about half ofthe patients with Hashimoto-associated encephalopathyhave nonenhancing MRI abnormalities with increased sig-nal intensity on T2-weighted images and on fast fluid-attenuated inversion recovery (FLAIR) [5]. While FLAIRis more sensitive than T2-weighted images for ischemia-induced cytotoxic oedema, diffusion-weighted imaging(DWI) is the most sensitive imaging sequence for ischaemicinjury. MR perfusion imaging is helpful for detecting arterialocclusion and hypoperfusion [3].Intravenous high-dose corticosteroid treatment is effec-tive, but sequelae are more common in children than inadults and most commonly include neuropsychological dif-ficulties, seizures and behaviour problems [4–6]. Non-response does not exclude Hashimoto enchephalopathy [7].In conclusion, Hashimoto thyroiditis needs to be con-sidered in children and adolescents with unexplained neuro-logical or psychiatric symptoms [8].References1. Watemberg N, Greenstein D, Levine A (2006) Encephalopathyassociated with Hashimoto thyroiditis: pediatric perspective. J ChildNeurol 21:1–52. Hoffmann F, Reiter K, Kluger G et al (2007) Seizures, psychosisand coma: Severe course of Hashimoto encephalopathy in a six-year-old girl. Neuropediatrics 38:197–1993. Barkovich AJ, Moore KR, Grant E et al. (2007) Pediatric neurora-diology. Elsevier Books, Salt Lake City, Utah4. Alink J, de Vries TW (2008) Unexplained seizures, confusion orhallucinations: think Hashimoto encephalopathy. Acta Paediatr97:451–4535. Grommes C, Griffin C, Downes K et al (2008) Steroid-responsiveencephalopathy associated with autoimmune thyroiditis presentingwith diffusion MR imaging changes. AJNR 8:1550–15516. Vasconcellos E, Piña-Garza JE, Fakhoury T et al (1999) Pediatricmanifestations of Hashimoto’s encephalopathy. Pediatr Neurol20:394–3987. Berger I, Castiel Y, Dor T (2010) Paediatric Hashimoto encepha-lopathy, refractory epilepsy and immunoglobulin treatment—unusual case report and review of the literature. Acta Paediatr99:1903–19058. Castro-Gago M, Gómez-Lado C, Maneiro-Freire M et al (2010)Hashimoto encephalopathy in a preschool girl. Pediatr Neurol42:143–146Pediatr Radiol (2012) 42:891–893 893

Steroid-responsive encephalopathy associated with Hashimoto thyroiditis

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Steroid-responsive encephalopathy associated with Hashimoto thyroiditis

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