|LETTER TO THE EDITOR
|Year : 2021 | Volume
| Issue : 2 | Page : 108-109
Hashimoto's encephalopathy presenting with cognitive impairment and vertical supranuclear gaze palsy
Abhishek Juneja1, Kuljeet Singh Anand2
1 Department of Neurology, Maharaja Agrasen Hospital, New Delhi, India
2 Department of Neurology, Dr. RML Hospital, Delhi, India
|Date of Submission||12-Jan-2021|
|Date of Acceptance||19-Jan-2021|
|Date of Web Publication||26-Mar-2021|
Dr. Abhishek Juneja
A-15, Old Quarters, Ramesh Nagar, New Delhi - 110 015
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Juneja A, Anand KS. Hashimoto's encephalopathy presenting with cognitive impairment and vertical supranuclear gaze palsy. Indian J Med Spec 2021;12:108-9
|How to cite this URL:|
Juneja A, Anand KS. Hashimoto's encephalopathy presenting with cognitive impairment and vertical supranuclear gaze palsy. Indian J Med Spec [serial online] 2021 [cited 2021 Apr 14];12:108-9. Available from: http://www.ijms.in/text.asp?2021/12/2/108/312218
We report a case of a 56-year-old male patient with progressive cognitive impairment for 4 months and frequent falls when walking for 2 weeks without a preceding history of headache, fever, vision loss, seizures, or head trauma. The patient had subacute onset, progressive cognitive impairment with difficulty carrying out complex motor tasks and loss of interest in surrounding activities. There was no medical history of diabetes, hypertension, thyroid illness, substance abuse, medication use, or cerebrovascular accident in the past. On examination, the vitals were normal, and the pupils were normal in size, and reaction to light, with bilateral vertical upgaze restriction that could be overcome by oculocephalic maneuver. A Mini-Mental State Examination (MMSE) resulted in 18/30 points. Sensorimotor examination was normal with bilateral flexor plantar response. Gait was cautious with tendency to fall backward due to restricted ocular movements. Routine blood investigations including blood counts, hepatic, renal, and glycemic profile were within normal limits. Magnetic resonance imaging of the brain and cerebrospinal fluid examination were unremarkable. Electroencephalogram showed generalized slow background activity without any epileptiform discharges. Thyroid function tests suggested normal serum thyroxin and triiodothyronine levels and elevated serum thyroid-stimulating hormone (TSH) levels (12.4 mIU/L, N: 0.4–4.0 mIU/L). Serum thyroid peroxidase (TPO) antibody levels were also elevated (960 IU/ml, N <35 IU/ml). We treated the patient with intravenous methylprednisolone at a dose of 1 g/day for 5 days, followed by tapering dose of oral prednisolone, keeping the possible diagnosis of Hashimoto's encephalopathy (HE). Thyroxin replacement therapy with 75 μg of levothyroxine was also initiated. The patient improved significantly over the next 4 weeks. The patient could score 24/30 on repeat MMSE after 4 weeks with significant improvement in gait and ocular motility; improvement in TSH (8.2 mIU/L) and anti-TPO (516 IU/ml) levels was also seen.
Hypothyroidism may cause various neurological manifestations and complications, involving both central nervous system (CNS) and peripheral nervous system. They include headache, ophthalmoplegia, cognitive impairment, psychosis, encephalopathy, myopathy including Hoffman's syndrome, and peripheral and entrapment neuropathy., HE is a rare neurological complication of autoimmune hypothyroidism. The diagnostic criteria for HE include (a) acute or subacute onset of altered mental status (AMS), (b) elevated antithyroid antibodies, (c) rapid response in mental status with corticosteroids, and (d) absence of structural, infectious, or other metabolic factors, which could explain the AMS and its response to steroids. The pathological mechanism of HE is not entirely clear. Vasculitis has been proposed as a possible mechanism. There have been very few reports of HE presenting with external ophthalmoplegia and cognitive impairment. We ruled out other causes of cognitive impairment with vertical gaze palsy like neurodegenerative disorders (e.g., progressive supranuclear palsy) and CNS infections (e.g., tuberculosis and Whipple's disease) by history and relevant investigations. We conclude that HE should be kept as differential diagnosis in patients presenting with rapidly progressive dementia with ophthalmoplegia, considering its treatable nature.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Stasiolek M. Neurological symptoms and signs in thyroid disease. Thyroid Res 2015;8:A25.
Juneja A, Anand K, Agrawal A. A unique presentation of Hashimoto's encephalopathy. Thyroid Res Pract 2020;17:94-5. [Full text]
Olmez I, Moses H, Sriram S, Kirshner H, Lagrange AH, Pawate S. Diagnostic and therapeutic aspects of Hashimoto's encephalopathy. J Neurol Sci 2013;331:67-71.
Nolte KW, Unbehaun A, Sieker H, Kloss TM, Paulus W. Hashimoto encephalopathy: A brainstem vasculitis? Neurology 2000;54:769-70.
Tafakhori A, Siroos B, Ghabaii M, Harirchain MH, Tajdini M, Garg SK. Hashimoto encephalopathy in case of progressive cognitive impairment; a case report. Emerg (Tehran) 2014;2:144-6.