Case Report
Reversible splenial lesion in auto-immune thyroid disease: a case report J. De Greef1, C. Jaumotte1, B. Quivron2, G. Derue1 1
Department of Internal Medicine, Centre Hospitalier Jolimont-Lobbes, Haine-Saint-Paul, Belgium, 2Department of Neurology, Centre Hospitalier Jolimont-Lobbes, Haine-Saint-Paul, Belgium Reversible lesions of the splenium of the corpus callosum constitute a clinicoradiological syndrome that has been associated to various medical conditions. We report the case of a 47-year-old man who presented with encephalopathy associated to auto-immune thyroid disease in which a reversible splenial lesion was isolated. Although encephalopathy associated to auto-immune thyroid disease is characterized by variable radiological findings, it has only been once associated with a reversible splenial lesion.
Keywords: Thyroiditis, Encephalopathy associated with autoimmune thyroid disease, Reversible lesion of the splenium of the corpus callosum
Introduction Encephalopathy associated with autoimmune thyroid disease (EAATD), also called Hashimoto’s encephalopathy, is a rare neurological condition that may affect patients with autoimmune thyroid diseases of any kind. It presents with a large spectrum of neurological or psychiatric conditions, high levels of anti-thyroid antibodies in blood, increased cerebrospinal fluid (CSF) protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to corticosteroids. In some cases, it is associated with mild lymphocytic pleocytosis.1,2
Case Report In May 2011, a 47-year-old man of Chinese origin presented at the emergency department with recent weight loss, headache, fever, and one episode of vomiting. He had a past medical history of Grave’s disease for which he had stopped follow-up and treatment several years ago. Initial clinical examination showed a body temperature of 39.3uC and a poor general condition. Neurological examination was normal. Blood tests revealed a severe hyperthyroidism with thyroid stimulating hormone ,0.01 mIU/ml and free-T4 .77.0 pg/ml, all routine tests being normal. Cerebral CT scanner was unrevealing, and CSF examination showed a mild lymphocytic pleocytosis (134 white cells/ml of which 96% were lymphocytes), with an increased protein concentration of 0.82 g/l, a high lactate concentration of 4.71 mmol/l, and a normal glucose concentration. Correspondence to: J. De Greef, Department of Internal Medicine, Hopital de Jolimont, rue Ferrer 159, 7100 Haine-Saint-Paul, Belgium. Email: [email protected]
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ß Acta Clinica Belgica 2014 DOI 10.1179/2295333713Y.0000000002
Bacterial smears, cultures, and Herpes Simplex PCR were negative on CSF. Thiamazol and propranolol were started to control hyperthyroidism, and the patient was admitted in the medical ward. Three days later, his consciousness severely deteriorated to a level of 10 (E4V2M4) on the Glasgow Coma Scale and he presented a urinary retention. Ceftriaxone, ampicillin, and aciclovir were started empirically. A new CSF collection confirmed the previous lymphocytic pleocytosis and high protein level. Antithyroid antibodies (anti-thyroid peroxidase and anti-thyroid stimulating hormone receptor) were found positive in the blood. A MRI study demonstrated an isolated lesion of the splenium of the corpus callosum, which was markedly hyperintense on T2- and hypointense on T1-weighted images, with no enhancement after contrast product injection (Fig. 1). Those findings were suggestive of reversible splenial lesion, a welldescribed clinicoradiological syndrome, indicating us to start the administration of corticoids, as all other investigations remain negative. The patient clinically improved, and all antibiotics were promptly stopped. A follow-up MRI, performed 14 days after the initial one, showed a regression of high signals along with a normalisation of apparent diffusion coefficient reductions. The patient remained asymptomatic at one year follow-up.
Discussion EAATD is generally associated with normal or nonspecific radiological findings. Ischaemic-like changes, demyelination, vasogenic oedema, and atrophy have been reported.3 We report a case in which EAATD is associated with a reversible lesion of the splenium of
Acta Clinica Belgica
2014
VOL.
69
NO.
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De Greef et al.
Encephalopathy and auto-immune thyroid disease
Figure 1 Isolated lesion of the splenium of the corpus callosum, with increased intensity on sagittal T2 image (A) and axial FLAIR image (B).
the corpus callosum. To the best of our knowledge, EAATD has only once been associated to a reversible splenial lesion, which disappeared completely with high doses of corticoids.3 The differential diagnosis of a reversible lesion of the splenium of the corpus callosum includes trauma, seizure, ischaemia, renal failure, hypoglycaemia, hypoor hypernatremia, alcohol, malnutrition, Marchiafava– Binagmi disease, viral encephalitis, altitude sickness, hypertension or preeclampsia, neoplasia, radiation therapy, chemotherapy, leukodystrophy, and extrapontine myelinolysis.4 Furthermore, we found two cases where it was linked to thyrotoxic encephalopathy.5 Independently of the origin, reversible splenial lesions have been recognized as constituting a new clinico-radiological syndrome associating clinically reversible symptoms to radiologically transient lesions of the splenium of the corpus callosum. Although the precise pathophysiological mechanism of this entity is still unknown, transient lesions of the corpus callosum are thought to be the result of cytotoxic or vasogenic oedema secondary to different aetiologies. This is
consistent with EAATD, where cytotoxic oedema rather than vasogenic oedema is thought to be at the origin of a transient ischaemic process. In conclusion, we hypothesized that the clinical condition of our patient could be attributed to a transient splenial lesion of the corpus callosum, secondary to an EAATD. A vasculitic process with subsequent ischaemia, as suspected in EAATD, is a plausible cause for transient splenial lesions.
References 1 Brain L, Jellinek EH, Ball K. Hashimoto’s disease and encephalopathy. Lancet. 1966;2:512–4. 2 Tamagno G, Celik Y, Simo´ R, Dihne´ M, Kimura K, Gelosa G, et al. Encephalopathy associated with autoimmune thyroid disease in patients with Graves’ disease: clinical manifestations, follow-up, and outcomes. BMC Neurol. 2010;10:27. 3 Chen N, Qin W, Wei C, Wang X, Li K. Time course of Hashimoto’s encephalopathy revealed by MRI: report of two cases. J Neurol Sci. 2011;300:169–72. 4 Doherty MJ, Jayadev S, Watson NF, Konchada RS, Hallam DK. Clinical implications of splenium magnetic resonance imaging signal changes. Arch Neurol. 2005;62:433–7. 5 Park MH, Ryu JK, Seo JA. Reversible splenial abnormality in thyrotoxic encephalopathy. Eur J Neurol. 2007;14:e23–4.
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Reversible splenial lesion in auto-immune thyroid disease: a case report J. De Greef1, C. Jaumotte1, B. Quivron2, G. Derue1 1
Department of Internal Medicine, Centre Hospitalier Jolimont-Lobbes, Haine-Saint-Paul, Belgium, 2Department of Neurology, Centre Hospitalier Jolimont-Lobbes, Haine-Saint-Paul, Belgium Reversible lesions of the splenium of the corpus callosum constitute a clinicoradiological syndrome that has been associated to various medical conditions. We report the case of a 47-year-old man who presented with encephalopathy associated to auto-immune thyroid disease in which a reversible splenial lesion was isolated. Although encephalopathy associated to auto-immune thyroid disease is characterized by variable radiological findings, it has only been once associated with a reversible splenial lesion.
Keywords: Thyroiditis, Encephalopathy associated with autoimmune thyroid disease, Reversible lesion of the splenium of the corpus callosum
Introduction Encephalopathy associated with autoimmune thyroid disease (EAATD), also called Hashimoto’s encephalopathy, is a rare neurological condition that may affect patients with autoimmune thyroid diseases of any kind. It presents with a large spectrum of neurological or psychiatric conditions, high levels of anti-thyroid antibodies in blood, increased cerebrospinal fluid (CSF) protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to corticosteroids. In some cases, it is associated with mild lymphocytic pleocytosis.1,2
Case Report In May 2011, a 47-year-old man of Chinese origin presented at the emergency department with recent weight loss, headache, fever, and one episode of vomiting. He had a past medical history of Grave’s disease for which he had stopped follow-up and treatment several years ago. Initial clinical examination showed a body temperature of 39.3uC and a poor general condition. Neurological examination was normal. Blood tests revealed a severe hyperthyroidism with thyroid stimulating hormone ,0.01 mIU/ml and free-T4 .77.0 pg/ml, all routine tests being normal. Cerebral CT scanner was unrevealing, and CSF examination showed a mild lymphocytic pleocytosis (134 white cells/ml of which 96% were lymphocytes), with an increased protein concentration of 0.82 g/l, a high lactate concentration of 4.71 mmol/l, and a normal glucose concentration. Correspondence to: J. De Greef, Department of Internal Medicine, Hopital de Jolimont, rue Ferrer 159, 7100 Haine-Saint-Paul, Belgium. Email: [email protected]
208
ß Acta Clinica Belgica 2014 DOI 10.1179/2295333713Y.0000000002
Bacterial smears, cultures, and Herpes Simplex PCR were negative on CSF. Thiamazol and propranolol were started to control hyperthyroidism, and the patient was admitted in the medical ward. Three days later, his consciousness severely deteriorated to a level of 10 (E4V2M4) on the Glasgow Coma Scale and he presented a urinary retention. Ceftriaxone, ampicillin, and aciclovir were started empirically. A new CSF collection confirmed the previous lymphocytic pleocytosis and high protein level. Antithyroid antibodies (anti-thyroid peroxidase and anti-thyroid stimulating hormone receptor) were found positive in the blood. A MRI study demonstrated an isolated lesion of the splenium of the corpus callosum, which was markedly hyperintense on T2- and hypointense on T1-weighted images, with no enhancement after contrast product injection (Fig. 1). Those findings were suggestive of reversible splenial lesion, a welldescribed clinicoradiological syndrome, indicating us to start the administration of corticoids, as all other investigations remain negative. The patient clinically improved, and all antibiotics were promptly stopped. A follow-up MRI, performed 14 days after the initial one, showed a regression of high signals along with a normalisation of apparent diffusion coefficient reductions. The patient remained asymptomatic at one year follow-up.
Discussion EAATD is generally associated with normal or nonspecific radiological findings. Ischaemic-like changes, demyelination, vasogenic oedema, and atrophy have been reported.3 We report a case in which EAATD is associated with a reversible lesion of the splenium of
Acta Clinica Belgica
2014
VOL.
69
NO.
3
De Greef et al.
Encephalopathy and auto-immune thyroid disease
Figure 1 Isolated lesion of the splenium of the corpus callosum, with increased intensity on sagittal T2 image (A) and axial FLAIR image (B).
the corpus callosum. To the best of our knowledge, EAATD has only once been associated to a reversible splenial lesion, which disappeared completely with high doses of corticoids.3 The differential diagnosis of a reversible lesion of the splenium of the corpus callosum includes trauma, seizure, ischaemia, renal failure, hypoglycaemia, hypoor hypernatremia, alcohol, malnutrition, Marchiafava– Binagmi disease, viral encephalitis, altitude sickness, hypertension or preeclampsia, neoplasia, radiation therapy, chemotherapy, leukodystrophy, and extrapontine myelinolysis.4 Furthermore, we found two cases where it was linked to thyrotoxic encephalopathy.5 Independently of the origin, reversible splenial lesions have been recognized as constituting a new clinico-radiological syndrome associating clinically reversible symptoms to radiologically transient lesions of the splenium of the corpus callosum. Although the precise pathophysiological mechanism of this entity is still unknown, transient lesions of the corpus callosum are thought to be the result of cytotoxic or vasogenic oedema secondary to different aetiologies. This is
consistent with EAATD, where cytotoxic oedema rather than vasogenic oedema is thought to be at the origin of a transient ischaemic process. In conclusion, we hypothesized that the clinical condition of our patient could be attributed to a transient splenial lesion of the corpus callosum, secondary to an EAATD. A vasculitic process with subsequent ischaemia, as suspected in EAATD, is a plausible cause for transient splenial lesions.
References 1 Brain L, Jellinek EH, Ball K. Hashimoto’s disease and encephalopathy. Lancet. 1966;2:512–4. 2 Tamagno G, Celik Y, Simo´ R, Dihne´ M, Kimura K, Gelosa G, et al. Encephalopathy associated with autoimmune thyroid disease in patients with Graves’ disease: clinical manifestations, follow-up, and outcomes. BMC Neurol. 2010;10:27. 3 Chen N, Qin W, Wei C, Wang X, Li K. Time course of Hashimoto’s encephalopathy revealed by MRI: report of two cases. J Neurol Sci. 2011;300:169–72. 4 Doherty MJ, Jayadev S, Watson NF, Konchada RS, Hallam DK. Clinical implications of splenium magnetic resonance imaging signal changes. Arch Neurol. 2005;62:433–7. 5 Park MH, Ryu JK, Seo JA. Reversible splenial abnormality in thyrotoxic encephalopathy. Eur J Neurol. 2007;14:e23–4.
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