- Email: [email protected]
A young female with urinary retention – Hashimoto's Encephalopathy
YAJEM-56453; No of Pages 2 American Journal of Emergency Medicine xxx (2017) xxx–xxx
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
A young female with urinary retention – A case report of Hashimoto's Encephalopathy Benjamin L. Cooper, MD a,⁎, Shane E. Appel, BS b, Hussam M. Ammar, MD c a b c
McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth), Department of Emergency Medicine, 6431 Fannin Street, JJL 445, Houston, TX 77030, United States McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth), United States McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth), Department of Internal Medicine, United States
a r t i c l e
i n f o
Article history: Received 18 January 2017 Accepted 25 January 2017 Available online xxxx Keywords: Hashimoto's Encephalopathy Topic: Hashimoto's thyroiditis Nystagmus Urinary retention
a b s t r a c t Hashimoto's Encephalopathy (HE) is a rare form of autoimmune encephalopathy associated with Hashimoto's thyroiditis in which patients experience cognitive impairment and various neurologic symptoms. We present a case of a young female that presented to the emergency department with urinary retention, and was ultimately diagnosed with HE. Examination was significant for direction-changing and vertical nystagmus (directionchanging nystagmus describes a phenomenon where the fast beat changes with the direction of gaze), hyperreflexia, clonus, and Babinski and Hoffman's reflexes (all upper motor neuron (UMN) signs). Her symptoms improved with high dose intravenous steroid administration. To our knowledge, urinary retention in the presence of other UMN signs has not been reported in association with HE; nor has this patient's type of nystagmus. Emergency physicians should be aware of this condition and can play an important role by considering it when neurologic findings are not explained by infectious, toxic, metabolic, or structural etiologies. Although relatively rare, if detected and treated early HE can have a good prognosis. © 2017 Elsevier Inc. All rights reserved.
1. Introduction Hashimoto's Encephalopathy (HE) is a rare form of autoimmune encephalopathy associated with Hashimoto's thyroiditis (HT). The disease entity was first described in 1966 by Brain et al. [1] and has since been reported in over 105 cases with an estimated prevalence of 2.1/100,000 [2,3]. HE can be summarized as a disease with the following features: (1) a pleomorphic clinical presentation with some degree of cognitive impairment and abnormal neurologic signs or symptoms that is usually relapsing and remitting (Table 1); (2) the presence of thyroperoxidase antibodies (TPOAb); (3) the absence of infectious, toxic, metabolic, neoplastic, paraneoplastic, or structural etiology; and (4) a response to corticosteroid treatment [4,5]. Although relatively rare, if detected and treated early HE can have a good prognosis [7,8]. 2. Case report A 26 year old female presented with urinary retention for one day. She presented two months postpartum after the successful caesarian delivery of a healthy baby. Upon further questioning, it was revealed that she had developed difficulty ambulating over the prior week prompting the use of a cane – a fact that she strangely seemed unalarmed by. Curiously, she had a similar problem one year prior that resolved with steroids when she was again two months postpartum after a spontaneous ⁎ Corresponding author. E-mail address: [email protected] (B.L. Cooper).
abortion, at which point she was diagnosed with HT – the details of which, including her diagnosis at the time, she was unable to recall. Physical examination revealed a young healthy appearing female in no distress with a pulse of 113, blood pressure of 155/117, respiratory rate 20, oxygen saturation 100%, and temperature 97.5 °F. On neurologic examination, she seemed to have some cognitive impairment manifesting as memory difficulties, but was alert and oriented. Other significant neurologic findings included direction-changing and vertical nystagmus, Hoffman's reflex of the right index finger, 4/5 strength to the right lower extremity proximal and distal muscles, bilateral positive Babinski reflexes, brisk 3 + knee/ankle reflexes to the bilateral lower extremities, and 10 beats of clonus provoked to the right lower extremity. A urinary catheter was placed evacuating 1000 mL of clear urine. Initial laboratory work up was significant for a white blood cell count of 13.8 thousand/μL, electrolytes within normal limits, a normal thyroid stimulating hormone (0.47 μIU/mL; reference range (RR) 0.36–3.74), and a negative urine toxicology screen. An MRI with and without contrast of the brain and full spine was unremarkable. The patient was subsequently admitted and underwent an eleven-day hospital course. A lumbar puncture was performed and cerebrospinal fluids (CSF) analysis revealed a normal opening pressure, 40 white blood cells/μL in the setting of 18,400 red blood cells/μL (appropriate pleocytosis given the traumatic nature of the procedure), elevated protein (57.5 mg/dL; RR 15–45), normal glucose, and elevated immunoglobulin G (IgG) (11.50 mg/dL; RR 0.00–3.40) without oligoclonal bands. Blood, urine, CSF cultures, and the CSF venereal disease research laboratory test (VDRL) were all ultimately negative. Anti-thyroperoxidase
http://dx.doi.org/10.1016/j.ajem.2017.01.054 0735-6757/© 2017 Elsevier Inc. All rights reserved.
Please cite this article as: Cooper BL, et al, A young female with urinary retention – A case report of Hashimoto's Encephalopathy, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.01.054
2
B.L. Cooper et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx Table 1 Neurologic manifestations of HE and their associated prevalences [2,3,4,6]. Neurologic symptom
Prevalence
Tremor Headache Transient aphasia Seizures Altered consciousness Focal deficits Sleep abnormalities Myoclonus Cognitive impairment Gait ataxia Status epilepticus
28–84% 13–50% 73–80% 52–66% 36–85% 18–31% 55% 37–65% 36–100% 28–65% 12%
antibodies (TPOAb) levels were elevated (123 IU/mL; RR b 34), and antithyroglobulin antibodies (TgAb) were negative. A host of other autoimmune assays including antinuclear antibodies, anti-SSA/B, anti-dsDNA, MPO-ANCA, and PR3-ANCA were ultimately negative or indeterminate (in the case of MPO-ANCA). Additionally, a paraneoplastic panel was unremarkable. By hospital day seven, the patient was started on 1000 mg of intravenous methylprednisolone daily for a three-day duration, which improved her symptoms; however, she still required a cane for ambulation at the time of discharge. One week after discharge, her symptoms completely resolved. 3. Discussion Several diagnostic criteria have been proposed, but no consensus within the neurologic and endocrine communities exists [4]. Generally, the diagnosis of HE is made when otherwise unexplained neurologic maladies exist with the presence of TPOAb (86–100%) or TgAb (73%) [4]. Patients most often present with subclinical hypothyroidism (35%) or euthyroidism (30%) as with our patient [3]. There appears to be no specific correlation between the severity of thyroid dysfunction and the severity of the patient's neurological symptoms [9]. HE affects women more frequently than men by a ratio of 4:1 [3]. 60–85% of patients have elevated CSF protein, 14% have elevated IgG, and most do not have oligoclonal bands (8–33%) [2,3,4]. Neuroimaging is helpful
only to exclude structural, neoplastic, or demyelinating conditions and non-specific findings can be seen in 36–49% of patients [2,3,4]. The pathophysiology is unclear, and the role of anti-thyroid antibodies is unknown [10]. Standard treatment is high-dose steroid therapy usually followed by a slow taper. A typical regimen would be intravenous methylprednisolone 1000 mg/day for three to seven days followed by oral prednisone 1–2 mg/kg/day with a slow taper [3,4]. Symptom response is variable, but nearly all patients with HE will respond within one week of initiating steroids [2,3,7]. This appears to be the first documented case of HE where urinary retention and UMN signs were present, and also the first report of serial HE relapses in the postpartum period [11]. Nystagmus is an infrequently reported finding, although it has been reported in a series of patients with predominately ataxic-type HE [12].
References [1] Brain L, Jellinek EH, Ball K. Hashimoto's disease and encephalopathy. Lancet 1966; 2(7462):512–4. [2] Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol 2003;60(2):164–71. [3] Marshall GA, Doyle JJ. Long-term treatment of Hashimoto's encephalopathy. J Neuropsychiatry Clin Neurosci 2006;18(1):14–20. [4] Montagna G, Imperiali M, Agazzi P, et al. Hashimoto's encephalopathy: a rare proteiform disorder. Autoimmun Rev 2016;15(5):466–76. [5] Castillo P, Woodruff B, Caselli R, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 2006;63(2):197–202. [6] Koros C, Economou A, Mastorakos G, Bonakis A, Kalfakis N, Papageorgiou SG. A selective memory deficit caused by autoimmune encephalopathy associated with Hashimoto thyroiditis. Cogn Behav Neurol 2012;25(3):144–8. [7] Olmez I, Moses H, Sriram S, Kirshner H, Lagrange AH, Pawate S. Diagnostic and therapeutic aspects of Hashimoto's encephalopathy. J Neurol Sci 2013;331(1–2):67–71. [8] Shaw PJ, Walls TJ, Newman PK, Cleland PG, Cartlidge NE. Hashimoto's encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers—report of 5 cases. Neurology 1991;41(2 (Pt 1)):228–33. [9] Fatourechi V. Hashimoto's encephalopathy: myth or reality? An endocrinologist's perspective. Best Pract Res Clin Endocrinol Metab 2005;19(1):53–66. [10] Schiess N, Pardo CA. Hashimoto's encephalopathy. Ann N Y Acad Sci 2008;1142: 254–65. [11] Sellal F, Berton C, Andriantseheno M, Clerc C. Hashimoto's encephalopathy: exacerbations associated with menstrual cycle. Neurology 2002;59(10):1633–5. [12] Matsunaga A, Ikawa M, Fujii A, Nakamoto Y, Kuriyama M, Yoneda M. Hashimoto's encephalopathy as a treatable adult-onset cerebellar ataxia mimicking spinocerebellar degeneration. Eur Neurol 2013;69(1):14–20.
Please cite this article as: Cooper BL, et al, A young female with urinary retention – A case report of Hashimoto's Encephalopathy, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.01.054
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
A young female with urinary retention – A case report of Hashimoto's Encephalopathy Benjamin L. Cooper, MD a,⁎, Shane E. Appel, BS b, Hussam M. Ammar, MD c a b c
McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth), Department of Emergency Medicine, 6431 Fannin Street, JJL 445, Houston, TX 77030, United States McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth), United States McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth), Department of Internal Medicine, United States
a r t i c l e
i n f o
Article history: Received 18 January 2017 Accepted 25 January 2017 Available online xxxx Keywords: Hashimoto's Encephalopathy Topic: Hashimoto's thyroiditis Nystagmus Urinary retention
a b s t r a c t Hashimoto's Encephalopathy (HE) is a rare form of autoimmune encephalopathy associated with Hashimoto's thyroiditis in which patients experience cognitive impairment and various neurologic symptoms. We present a case of a young female that presented to the emergency department with urinary retention, and was ultimately diagnosed with HE. Examination was significant for direction-changing and vertical nystagmus (directionchanging nystagmus describes a phenomenon where the fast beat changes with the direction of gaze), hyperreflexia, clonus, and Babinski and Hoffman's reflexes (all upper motor neuron (UMN) signs). Her symptoms improved with high dose intravenous steroid administration. To our knowledge, urinary retention in the presence of other UMN signs has not been reported in association with HE; nor has this patient's type of nystagmus. Emergency physicians should be aware of this condition and can play an important role by considering it when neurologic findings are not explained by infectious, toxic, metabolic, or structural etiologies. Although relatively rare, if detected and treated early HE can have a good prognosis. © 2017 Elsevier Inc. All rights reserved.
1. Introduction Hashimoto's Encephalopathy (HE) is a rare form of autoimmune encephalopathy associated with Hashimoto's thyroiditis (HT). The disease entity was first described in 1966 by Brain et al. [1] and has since been reported in over 105 cases with an estimated prevalence of 2.1/100,000 [2,3]. HE can be summarized as a disease with the following features: (1) a pleomorphic clinical presentation with some degree of cognitive impairment and abnormal neurologic signs or symptoms that is usually relapsing and remitting (Table 1); (2) the presence of thyroperoxidase antibodies (TPOAb); (3) the absence of infectious, toxic, metabolic, neoplastic, paraneoplastic, or structural etiology; and (4) a response to corticosteroid treatment [4,5]. Although relatively rare, if detected and treated early HE can have a good prognosis [7,8]. 2. Case report A 26 year old female presented with urinary retention for one day. She presented two months postpartum after the successful caesarian delivery of a healthy baby. Upon further questioning, it was revealed that she had developed difficulty ambulating over the prior week prompting the use of a cane – a fact that she strangely seemed unalarmed by. Curiously, she had a similar problem one year prior that resolved with steroids when she was again two months postpartum after a spontaneous ⁎ Corresponding author. E-mail address: [email protected] (B.L. Cooper).
abortion, at which point she was diagnosed with HT – the details of which, including her diagnosis at the time, she was unable to recall. Physical examination revealed a young healthy appearing female in no distress with a pulse of 113, blood pressure of 155/117, respiratory rate 20, oxygen saturation 100%, and temperature 97.5 °F. On neurologic examination, she seemed to have some cognitive impairment manifesting as memory difficulties, but was alert and oriented. Other significant neurologic findings included direction-changing and vertical nystagmus, Hoffman's reflex of the right index finger, 4/5 strength to the right lower extremity proximal and distal muscles, bilateral positive Babinski reflexes, brisk 3 + knee/ankle reflexes to the bilateral lower extremities, and 10 beats of clonus provoked to the right lower extremity. A urinary catheter was placed evacuating 1000 mL of clear urine. Initial laboratory work up was significant for a white blood cell count of 13.8 thousand/μL, electrolytes within normal limits, a normal thyroid stimulating hormone (0.47 μIU/mL; reference range (RR) 0.36–3.74), and a negative urine toxicology screen. An MRI with and without contrast of the brain and full spine was unremarkable. The patient was subsequently admitted and underwent an eleven-day hospital course. A lumbar puncture was performed and cerebrospinal fluids (CSF) analysis revealed a normal opening pressure, 40 white blood cells/μL in the setting of 18,400 red blood cells/μL (appropriate pleocytosis given the traumatic nature of the procedure), elevated protein (57.5 mg/dL; RR 15–45), normal glucose, and elevated immunoglobulin G (IgG) (11.50 mg/dL; RR 0.00–3.40) without oligoclonal bands. Blood, urine, CSF cultures, and the CSF venereal disease research laboratory test (VDRL) were all ultimately negative. Anti-thyroperoxidase
http://dx.doi.org/10.1016/j.ajem.2017.01.054 0735-6757/© 2017 Elsevier Inc. All rights reserved.
Please cite this article as: Cooper BL, et al, A young female with urinary retention – A case report of Hashimoto's Encephalopathy, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.01.054
2
B.L. Cooper et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx Table 1 Neurologic manifestations of HE and their associated prevalences [2,3,4,6]. Neurologic symptom
Prevalence
Tremor Headache Transient aphasia Seizures Altered consciousness Focal deficits Sleep abnormalities Myoclonus Cognitive impairment Gait ataxia Status epilepticus
28–84% 13–50% 73–80% 52–66% 36–85% 18–31% 55% 37–65% 36–100% 28–65% 12%
antibodies (TPOAb) levels were elevated (123 IU/mL; RR b 34), and antithyroglobulin antibodies (TgAb) were negative. A host of other autoimmune assays including antinuclear antibodies, anti-SSA/B, anti-dsDNA, MPO-ANCA, and PR3-ANCA were ultimately negative or indeterminate (in the case of MPO-ANCA). Additionally, a paraneoplastic panel was unremarkable. By hospital day seven, the patient was started on 1000 mg of intravenous methylprednisolone daily for a three-day duration, which improved her symptoms; however, she still required a cane for ambulation at the time of discharge. One week after discharge, her symptoms completely resolved. 3. Discussion Several diagnostic criteria have been proposed, but no consensus within the neurologic and endocrine communities exists [4]. Generally, the diagnosis of HE is made when otherwise unexplained neurologic maladies exist with the presence of TPOAb (86–100%) or TgAb (73%) [4]. Patients most often present with subclinical hypothyroidism (35%) or euthyroidism (30%) as with our patient [3]. There appears to be no specific correlation between the severity of thyroid dysfunction and the severity of the patient's neurological symptoms [9]. HE affects women more frequently than men by a ratio of 4:1 [3]. 60–85% of patients have elevated CSF protein, 14% have elevated IgG, and most do not have oligoclonal bands (8–33%) [2,3,4]. Neuroimaging is helpful
only to exclude structural, neoplastic, or demyelinating conditions and non-specific findings can be seen in 36–49% of patients [2,3,4]. The pathophysiology is unclear, and the role of anti-thyroid antibodies is unknown [10]. Standard treatment is high-dose steroid therapy usually followed by a slow taper. A typical regimen would be intravenous methylprednisolone 1000 mg/day for three to seven days followed by oral prednisone 1–2 mg/kg/day with a slow taper [3,4]. Symptom response is variable, but nearly all patients with HE will respond within one week of initiating steroids [2,3,7]. This appears to be the first documented case of HE where urinary retention and UMN signs were present, and also the first report of serial HE relapses in the postpartum period [11]. Nystagmus is an infrequently reported finding, although it has been reported in a series of patients with predominately ataxic-type HE [12].
References [1] Brain L, Jellinek EH, Ball K. Hashimoto's disease and encephalopathy. Lancet 1966; 2(7462):512–4. [2] Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol 2003;60(2):164–71. [3] Marshall GA, Doyle JJ. Long-term treatment of Hashimoto's encephalopathy. J Neuropsychiatry Clin Neurosci 2006;18(1):14–20. [4] Montagna G, Imperiali M, Agazzi P, et al. Hashimoto's encephalopathy: a rare proteiform disorder. Autoimmun Rev 2016;15(5):466–76. [5] Castillo P, Woodruff B, Caselli R, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 2006;63(2):197–202. [6] Koros C, Economou A, Mastorakos G, Bonakis A, Kalfakis N, Papageorgiou SG. A selective memory deficit caused by autoimmune encephalopathy associated with Hashimoto thyroiditis. Cogn Behav Neurol 2012;25(3):144–8. [7] Olmez I, Moses H, Sriram S, Kirshner H, Lagrange AH, Pawate S. Diagnostic and therapeutic aspects of Hashimoto's encephalopathy. J Neurol Sci 2013;331(1–2):67–71. [8] Shaw PJ, Walls TJ, Newman PK, Cleland PG, Cartlidge NE. Hashimoto's encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers—report of 5 cases. Neurology 1991;41(2 (Pt 1)):228–33. [9] Fatourechi V. Hashimoto's encephalopathy: myth or reality? An endocrinologist's perspective. Best Pract Res Clin Endocrinol Metab 2005;19(1):53–66. [10] Schiess N, Pardo CA. Hashimoto's encephalopathy. Ann N Y Acad Sci 2008;1142: 254–65. [11] Sellal F, Berton C, Andriantseheno M, Clerc C. Hashimoto's encephalopathy: exacerbations associated with menstrual cycle. Neurology 2002;59(10):1633–5. [12] Matsunaga A, Ikawa M, Fujii A, Nakamoto Y, Kuriyama M, Yoneda M. Hashimoto's encephalopathy as a treatable adult-onset cerebellar ataxia mimicking spinocerebellar degeneration. Eur Neurol 2013;69(1):14–20.
Please cite this article as: Cooper BL, et al, A young female with urinary retention – A case report of Hashimoto's Encephalopathy, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.01.054