Hashimoto's encephalopathy mimicking ... - Springer Link

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Mar 23, 2011 - Dear Sirs,. A 39-year-old man was admitted for complaints of wors- ening gait difficulty, weakness and dysarthria. His symp- toms had ...
J Neurol (2011) 258:1705–1707 DOI 10.1007/s00415-011-5976-2

LETTER TO THE EDITORS

Hashimoto’s encephalopathy mimicking spinocerebellar ataxia Yi Tang • Changbiao Chu • Michael T. Lin • Gangzhi Wei • Xinqing Zhang • Yuwei Da • Hui Huang • Jianping Jia

Received: 10 January 2011 / Revised: 17 February 2011 / Accepted: 21 February 2011 / Published online: 23 March 2011 Ó Springer-Verlag 2011

Dear Sirs, A 39-year-old man was admitted for complaints of worsening gait difficulty, weakness and dysarthria. His symptoms had developed 17 months earlier with unsteadiness of gait. Subsequently, he gradually developed difficulties in handwriting, weakness and dysarthria. His symptoms were considered as spinocerebellar ataxia in a local hospital and treated with CoQ10, and vitamins B1 and B12. No improvement was observed. His disability accelerated 2 weeks prior to admission. There is no family history of cerebellar symptoms. Physical examination revealed palpable thyroid gland. Neurological examination showed normal mental status, right central facial weakness, lingual fasciculations, brisk jaw jerk, hyperactive gag reflex and scanning dysarthria. Strength of left upper and right limbs was 4/5 with bilateral palmomental reflexes and right Babinski signs. Focal dystonia was observed in the right hand. He was only able to walk several steps, with broad-based, ataxic gait. Marked

Y. Tang and C. Chu contributed equally to this work. Y. Tang  C. Chu  G. Wei  X. Zhang  Y. Da  J. Jia (&) Department of Neurology, Xuanwu Hospital, Capital Medical University, 45 Changchun Street, 100053 Beijing, Poeple’s Republic of China e-mail: [email protected] M. T. Lin Department of Neurology and Neuroscience, Weill Medical College of Cornell University/ NewYork-Presbyterian Hospital, New York, USA H. Huang Department of Cardiology, Sun Yat-sen Memorial Hospital of Sun Yat-sen University, Guangzhou, Poeple’s Republic of China

slowing and dysmetria of rapid alternating movements were noted. Pinprick and position sensations were preserved. There was no orthostatic hypotension. Magnetic resonance imaging (MRI) of the brain disclosed slight atrophy of the cerebellum without gadolinium enhancement (Fig. 1a). The electroencephalogram, Montreal Cognitive Assessment test and electromyography were normal. Cerebrospinal fluid (CSF) showed 126 mg/dl protein (normal 15–45 mg/dl) and normal IgG index. There were no oligoclonal bands (OB). Anti-GM1, GQ1b and GD1b antibodies were negative in both serum and CSF. Screenings of connective tissue disease, heavy metal and antibodies against viruses were negative. Angiotensin converting enzyme, vitamin B12, vitamin E, folate and rapid plasma reagin tests were unremarkable. Paraneoplastic antibodies were not detected in the serum. The chest CT was normal. Positron emission tomography (PET) only demonstrated a slightly decreased FDG uptake in the right thalamus (Fig. 1b). Genetic testing of SCA1-7, SCA12, SCA17 and DRPLA was negative. The patient was euthyroid with increased thyroglobulin antibody (TG-Ab) and thyroperoxidase antibody (TPO-Ab) concentrations at 83.2 U/ml (normal 0–60 U/ml) and [1,300 U/ml (normal 0–60 U/ml), respectively. Both TGAb and TPO-Ab were present in the CSF at 17.4 and 87.5 U/ml respectively. Thyroid ultrasound and scan revealed slightly enlarged thyroid gland with several hypoechoic areas and normal tracer uptake. Although antigliadin antibodies were not tested, neurological manifestations of gluten sensitivity are mainly ataxia, peripheral neuropathy, epilepsy and headache [6], which are not typical in this case. We diagnosed the condition as Hashimoto’s encephalopathy (HE) and initiated intravenous methylprednisolone 1 g/day for 5 days followed by oral prednisone tapering.

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J Neurol (2011) 258:1705–1707

Fig. 1 MRI and PET scans a MRI revealed slight atrophy of the cerebellum b PET scan showed slightly decreased FDG uptake in the right thalamus

His gait showed improvement 3 days later. On the seventh day he could walk 100 m by himself. His ataxia and weakness progressed again when oral prednisone was tapered to 40 mg/day. Prednisone was, therefore, increased to 50 mg/day and tapered more slowly. Two months later, he could walk 1,000 m alone, but speech was unchanged. HE, also termed steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), was initially described by Brain et al. [3] in 1966. The common characteristics were elevated antithyroid antibodies, various neurological deficits and responsiveness to steroid therapy [4, 5, 10]. Pathogenesis is still not clear. In nearly all HE case reports, steroids improved the symptoms. Plasmapheresis and intravenous immunoglobulins were also reported helpful [2, 7]. Gait ataxia is a common feature in HE, as high as 65% in a series report [4]. However, cerebellar symptoms were rarely reported as the predominant symptoms with a subacute or acute course [8, 9]. Selim et al. [11] also reported another type of Hashimoto’s thyroiditis associated cerebellar ataxia: progressive non-familial adult onset cerebellar degeneration (PNACD), in which, unlike this case, cerebellar atrophy is prominent, and in one patient treated with prednisone, no significant beneficial effect was observed. The mechanism of cerebellar involvement in HE is not clear. Recently, Blanchin et al. [1] reported that TPO-Abs from HE patients were able to bind cerebellar cells. However, the role of TPO-Ab in the pathogenesis of HE remains uncertain [10].

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Another unique characteristic in this case is the absence of manifestations of encephalopathy, which appeared instead to be the clinical picture of spinocerebellar ataxia. Cognitive impairment and altered level of consciousness have been proposed as essential for the diagnosis of HE [4, 5]. However, in this case and the previous HE report [9], without encephalopathy, neurological deficits improved with steroid therapy. Considering the reversible course, these patients should not be excluded from having HE. The spectrum of HE could be quite heterogeneous and broader than previously thought. We recommend that HE should always be in the differential diagnosis while evaluating CNS disorders, including spinocerebellar ataxia. Acknowledgments This work was supported by the grants from the National Science Foundation of China (No. 30900478, 30973207), The New-Star of Science and Technology supported by Beijing Metropolis (No. 2010B053) and Beijing Natural Science Foundation (No. 7102072). Conflict of interest of interest.

The authors declare that they have no conflict

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