Hashimoto's encephalopathy with clinical features similar to those of Creutzfeldt-Jakob disease

Abstract

In 1966, Brain et al. described the first case of Hashimoto's encephalopathy in a patient with stroke-like episodes, seizure activities, and delirium. The symptoms were recurrent and reversible and were not associated with thyroid dysfunction 1 . Since then, Hashimoto's encephalopathy has been recognized as a rare condition associated to Hashimoto's thyroiditis and to the presence of high concentrations of antithyroid antibodies, unlike those described in myxedema and in thyrotoxicosis 2 . Two clinical forms have been described: the vasculitic form with stroke-like episodes, seizure activities, and slight cognitive decline; and the diffuse form with symptoms of depression, psychosis, myoclonus, tremors, delusion, fluctuations in the level of consciousness, and dementia. The clinical manifestations can be fluctuating or persistent, and reversible, if properly treated with immunosuppressive therapy 3 . In some cases, Hashimoto's encephalopathy can show rapidly progressive dementia, myoclonus, and electroencephalographic changes with periodic recurrence of the triphasic complexes similar to those of Creutzfeldt-Jakob disease, which makes a differential diagnosis essential to both pathologies 4 . We describe the case of a patient with Hashimoto's encephalopathy who showed clinical manifestations similar to those of Creutzfeldt-Jakob disease. The recognition of the condition was essential for the treatment and regression of the clinical presentation. CaSEA female patient, 68-years-old, Caucasian, widow, born in Rio de Janeiro, developed a clinical presentation of daily sadness, discouragement, decreased appetite, insomnia, and cognitive decline. The condition of the patient worsened with persecutory delusions, visual hallucinations, gait disturbance, tremor of the extremities, muscle spasms, and fluctuations in the level of consciousness. She had a history of hypothyroidism and did not use any medication. At admission, she was sleepy, disoriented, showing myoclonus, paratonia on the back of the head and arms, her deep reflexes were symmetrical with plantar flexion response and suction and grasping reflexes were present. The rest of the physical examination was normal.The laboratory exams, including a complete blood count; biochemistry; arterial gasometry; hepatic function tests; autoantibody tests, such as anti-nuclear factor (ANF), anti-Ro, antiLa, pANCA and cANCA, were either normal or negative. The lev-

Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies

“…[12–16] Various symptoms are used to define clinical subtypes of HE, such as the Creutzfeldt–Jakob disease-like form and the cerebellar ataxic form. [22–24] However, the diversity of symptoms and the high prevalence of antithyroid antibodies in healthy populations may make the diagnosis of HE difficult. We discovered anti-NAE antibodies, autoantibodies that specifically recognize only the NH 2 -terminal of α-enolase, in the serum of patients with HE by proteomic analyses and demonstrated a high prevalence and specificity of these antibodies in patients with HE.…”

Clinical findings of a probable case of MM2-cortical-type sporadic Creutzfeldt-Jakob disease with antibodies to anti-N-terminus of α-enolase

“…We reviewed 4 reported cases of CJD-type Hashimoto's encephalopathy and our case (Table 1). [7][8][9][10] Responsiveness to corticosteroids is a pivotal differentiator between CJDtype Hashimoto's encephalopathy and CJD with anti-NAE Abs. A work-up that includes thyroid hormone tests and anti-thyroid glands antibodies is a crucial first step diagnosing progressive dementia including Hashimoto's encephalopathy.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The MRI can also reveal acute infarcts, raising the specter of vasculitis [256]. The differential for SREAT includes viral encephalitis, Creutzfeldt-Jakob disease, and other forms of dementia [251,[257][258][259][260][261].…”