Umschriebene Vaskulitis mit Posteriorinfarkt bei Hashimoto-Enzephalopathie

Abstract

The aetiology of Hashimoto's encephalopathy is still unknown. A 52-year-old woman with so far unspecific thyroid disorder presented with acute onset of right-sided sensory loss and visual disturbances. The neurological examination revealed a right upper quadrant anopsia and subtle right-sided sensory loss and weakness. The cranial MRI showed a left-sided cerebral infarction. MR angiography demonstrated a stenosis of the proximal segment of the left posterior cerebral artery, which was confirmed by conventional catheter angiography. The patient had no cardiovascular risk factors, no signs of systemic vasculitis, and no thromboembolic disorder. Thyroid function tests showed a subclinical hypothyroidism with plasma TSH level of 12.0 mU/ml, and thyroid antibodies were markedly elevated (hTG-AB 3390 U/ml, TPO-AB > 8000 U/ml). Typical features of Hashimoto's disease were demonstrated by ultrasound and scintigraphic examination of the thyroid gland. To the best of our knowledge, this is the first description of Hashimoto encephalopathy with localised vasculitis of the posterior cerebral arteries and left posterior infarction. It could be shown that the MR angiogram is a feasible tool to demonstrate regression of the vasculitis under glucocorticoid therapy.

Hashimoto Encephalopathy (Autoimmune Encephalitis)

“…Initial images, particularly those from the left caudate nucleus and the periphery of the cerebellar hemisphere from the same side (being very suggestive of infarction), should possibly have led to steroid treatment being used from the beginning (because stroke-like vasculitis in Hashimoto encephalitis has already been described). 22,23 Remission of transitory ischemia-like events with steroid treatment in this patient and reports of cerebral necropsy or biopsy in several cases in the literature 22,24 would indicate autoimmune vasculitic phenomenon regarding the small vessels. 5,15,22,24 -28 It was remarkable, like in other cases in the literature, that initial MRI images after normalization of hypothyroidism (6 months) and after more than 1 year of corticotherapy were not modified.…”

Detection of thyroid peroxidase mRNA and protein in orbital tissue

“…How TPOAb might confer protection is unknown. TPOAb have been associated with a diverse range of non-thyroidal conditions including depression (13), infertility (14), Hashimoto's encephalopathy (15) and improved prognosis in breast cancer (16). Autoantibodies to TPO are polyclonal and are largely directed to a region on the native antigen, termed the immunodominant region (17,18).…”

Hashimoto-Enzephalopathie

“…Ursache der sowohl beobachteten fokalen als auch diffusen Hirnbeteiligung könnte eine zerebrale Immunkomplexvaskulitis sein [2,7]. Neben einer vaskulitischen wird in der Literatur eine diffus progressive Verlaufsform unterschieden, welche häufiger mit demenzieller Entwicklung und Anfällen einherzugehen scheint [1,3,11]. Die Hashimoto-Enzephalopathie wird vom Kindes-bis ins hohe Erwachsenenalter, bei Frauen häufiger als bei Männern beschrieben [2,3,9].…”

“…Bildgebende Untersuchungen tragen nicht immer zur Diagnose bei, da sie in bis zu 8 % Normalbefunde bzw. unspezifische Veränderungen zeigen [5,11]. Wie im beschriebenen Fall könnte jedoch die Hirnperfusions-SPECT pathologische Veränderungen aufdecken, welche mit der ätiologisch diskutierten vaskulitischen Verlaufsform übereinstimmen [11].…”

“…unspezifische Veränderungen zeigen [5,11]. Wie im beschriebenen Fall könnte jedoch die Hirnperfusions-SPECT pathologische Veränderungen aufdecken, welche mit der ätiologisch diskutierten vaskulitischen Verlaufsform übereinstimmen [11]. In der Literatur sind SPECT-Untersuchungen diesbezüglich bisher kaum berücksichtigt [1,12].…”