Correspondence Address: Dr. Niraj Kumar Department of Neurology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand. India
Source of Support: None, Conflict of Interest: None
Hashimoto’s encephalopathy (HE) commonly presents with two types of neurological presentation: recurrent stroke-like episodes or seizures and progressive cognitive decline. Being a treatable disorder, one cannot afford to miss the diagnosis. We report a female with HE who presented with a history of recurrent episodes of catatonia, recovering completely with steroids. The diagnosis of the primary disease got delayed for 5 months. HE presenting as catatonia has been reported rarely. A high degree of suspicion in appropriate clinical settings, resulting in early diagnosis, is rewarding as HE usually show good response to corticosteroids.
How to cite this article: Shree R, Madhaw G, Manchanda R, Radhakrishnan DM, Kumar N. Steroid responsive catatonia: A case of Hashimoto’s encephalopathy. Ann Mov Disord 2020;3:51-5
How to cite this URL: Shree R, Madhaw G, Manchanda R, Radhakrishnan DM, Kumar N. Steroid responsive catatonia: A case of Hashimoto’s encephalopathy. Ann Mov Disord [serial online] 2020 [cited 2022 Nov 30];3:51-5. Available from: https://www.aomd.in/text.asp?2020/3/1/51/281746
Hashimoto’s encephalopathy (HE) is a neurological disorder associated with high titers of thyroid antibodies and manifests with a wide range of neuropsychiatric features including seizures, altered sensorium, stroke-like episodes, impaired memory, and abnormal gait and movement disorders such as tremor. The disease is known as steroid-responsive encephalopathy associated with autoimmune thyroiditis because of remarkable recovery after steroids; however, alternate plasmapheresis, immunoglobulins, and other alternate immunomodulation have been tried in refractory cases. Catatonia as a presenting manifestation of HE is unusual. We report a 60-year-old woman presenting with recurrent episodes of catatonia for 5 months before being diagnosed with HE.
A 60-year-old woman presented with a 2-day history of acute-onset irrelevant speech and visual hallucinations followed by mutism, altered sensorium, swallowing difficulty, immobility, and generalized tremulousness in the absence of fever. She had two similar episodes of acute neurological illness in the past 5 months, both improving with symptomatic therapy from general physician. There was no pertinent family history. General examination revealed thyroid swelling in the neck. Neurological examination revealed catalepsy with waxy flexibility of limbs [Video 1], limb and truncal rigidity, brisk deep tendon jerks along with rest and action tremor, and myoclonus involving bilateral upper extremities. Her laboratory workup revealed increased erythrocyte sedimentation rate (ESR) of 80mm in the 1st h, and positive serum C-reactive protein (CRP). Remainder of the routine investigations including renal, liver, and thyroid function tests was unremarkable. Creatine phosphokinase level was elevated (398 U/L; normal <145 U/L). Magnetic resonance imaging (MRI) of brain failed to reveal any abnormality. Cerebrospinal fluid (CSF) examination revealed raised protein level at 166mg/dL (normal, 15–45mg/dL) with a cell count of 5/mm3 and glucose level of 100mg/dL with the corresponding random blood glucose being 120mg/dL. Her electroencephalogram (EEG) showed diffuse delta–theta range slowing with no epileptiform discharges [Figure 1B]. A possibility of acute catatonia resulting from possible organic encephalopathy was considered. Contrast-enhanced computed tomography (CT) of thorax, abdomen, and pelvis was nonconclusive. CT of neck revealed multinodular goiter, and anti-TPO (thyroid peroxidase) antibodies titer was significantly elevated to >1300 IU/mL (normal <60 IU/mL). Fine needle aspiration of thyroid gland revealed lymphocytic infiltration in follicular cells with anisonucleosis, suggestive of Hashimoto’s thyroiditis [Figure 1A]. The patient was started on intravenous methylprednisolone in a dose of 1g daily for 5 days followed by oral steroids. She made remarkable recovery [Video 1] within 1 week and reached premorbid state of functionality in 3 months.
Figure 1: (A) Smear examined from thyroid shows thyroid follicular cells (black arrow) of moderate cellularity, arranged in micro- and macro-follicular pattern with intense lymphocytic infiltration within the follicles (yellow arrow) (Giemsa stain, ×10 magnification). (B) EEG showing diffuse theta range slowing (background of 3–5 Hz) with no epileptiform discharges
Although heterogeneous features are common in HE, patients commonly may present either with an acute stroke-like neurological deficit or a progressive neurological decline with seizures, dementia, and psychosis. The onset of illness may be acute or subacute with either progressive or fluctuating symptoms. Diagnosis of HE should be considered in cases presenting with encephalopathy with seizures, myoclonus, hallucinations, or stroke-like episodes (common presentation) with subclinical or mild hypothyroidism and high titers of anti-TPO or thyroglobulin antibodies in serum in the absence of another pathology accounting for the clinical spectrum [Box 1].,, The presence of catalepsy, mutism, and immobility along with generalized tremulousness and myoclonic jerks in our case favored the possibility of catatonia. Although catatonia has been reported in only six cases of HE to date, it was not the presenting complaint in the described cases [Table 1].,,,,, Our case presented with catatonia as the major initial neurological manifestation. Interestingly, all the cases including ours were that of females. It may be accounted by the fact that HE predominantly involves females (female:male = 4:1).
Table 1: Reported cases of Hashimoto’s encephalopathy manifesting catatonia
Although association with high anti-TPO antibody titer forms a core diagnostic criterion for HE, the status of serum thyroid hormone profile is variable in these cases. Euthyroid state and subclinical hypothyroidism each are observed in a third of cases, with hypothyroidism in 20% and hyperthyroidism in 7%–8% cases. Our patient had euthyroid state. Nonspecific hematological abnormalities such as raised ESR and CRP have been reported in 15%–20% cases as were observed in our case too. CSF examination may reveal a cytoalbuminological dissociation in more than two-third cases of HE as was observed in index case.
MRI brain is usually normal or may show diffuse cerebral atrophy or nonspecific white matter hyperintensities, with the latter sometimes disappearing following treatment with steroids. MRI can mimic ischemic stroke or degenerative disease. Nonspecific EEG abnormalities are reported in 98% cases with diffuse slowing of the background commonly observed. Frontal rhythmic slowing, triphasic waves, and periodic sharp waves have occasionally been reported., The EEG of our case revealed diffuse slowing of the background in the delta–theta range.
Patients with HE show remarkable response with steroids; however, guidelines about the duration and follow-up investigations are lacking. Early initiation of therapy with either high-dose intravenous methylprednisolone (1g/day) for 5 days or oral prednisone (1mg/kg body weight per day) is beneficial. The clinical response should be closely monitored, and oral steroids should be tapered off slowly over weeks to months. Cases relapsing after tapering off steroids or those refractory to steroids may respond to cyclophosphamide, azathioprine, intravenous immunoglobulin, plasma exchange, or a combination of these therapies. Our case showed a dramatic response to intravenous methylprednisolone 1g/day for 5 days followed by oral prednisolone 1mg/kg/day for the next 3 months. The oral corticosteroids were gradually tapered off in subsequent 3 months.
HE frequently has a heterogeneous neurological presentation and should be suspected in cases presenting with acute or subacute encephalopathy and lacking an identifiable cause on the relevant investigations. Our case uncovers a very unusual presentation of HE in the form of catatonia with a dramatic response to steroids. Therefore, HE should be considered as a possible etiology of organic catatonia, especially in females, and a high degree of suspicion may facilitate early diagnosis and prompt therapy.
[TAG:2]Box 1: Major neurological manifestations of Hashimoto’s encephalopathy,,[/TAG:2]
Types of presentation:
Acute/vasculitis type: 25%
Diffuse progressive type: 75%
Major neurological features:
Cognitive impairment (84.6%)
Visual hallucination or paranoid ideation (36%)
Stroke-like features (27%)
Depressive disorder (23%)
Status epilepticus (12%)
Acute encephalopathy (10.9%)
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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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