pattismith
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2018
A novel assessment and treatment approach to patients with Hashimoto’s encephalopathy
Kate Laycock, Abhijit Chaudhuri, Charlotte Fuller, Zahra Khatami, Frederick Nkonge, and Nemanja Stojanovic
Barking, Havering and Redbridge University Hospitals NHS Trust, Queen's Hospital, Rom Valley Way, Romford, Essex, London, UK
Summary
Hashimoto’s encephalopathy (HE) is rarely reported with only a few hundred cases published. Diagnosis is made in patients with an appropriate clinical picture and high antithyroperoxidase (anti-TPO) antibodies after infectious, toxic and metabolic causes of encephalopathy have been excluded.
There is little objective data on the neurocognitive impairment in patients with HE and their improvement with treatment. We present the case of a 28-year-old woman with HE. Approach to management was novel as objective neuropsychological assessment was used to assess her clinical condition and response to treatment. Intravenous immunoglobulin (IVIg) as the first-line treatment instead of steroids. She responded well. The case illustrates that a different approach is required for the diagnosis and treatment of HE. A new diagnostic criteria is proposed that includes neurocognitive assessment, serum and CSF antibodies, an abnormal EEG and exclusion of other causes of encephalopathy. Furthermore, treatment should be tailored to the patient.
Learning points:
Background
Hashimoto’s encephalopathy (HE) is characterised by a subacute, steroid-responsive encephalopathy in patients with positive antithyroid antibodies (1). The clinical presentation is highly variable; common clinical features include confusion, altered consciousness, tremor, transient aphasia, gait ataxia, seizures and sleep abnormalities. The natural history of the disease is relapsing-remitting (2). There have been just a few hundred cases reported in the literature. The average age of onset is 42 years, and it has an estimated prevalence of 2.1/100 000 people (3). The heterogeneity of the clinical presentation, the high prevalence of antithyroid antibodies in the general population and the lack of a clear underlying pathophysiological process has led many to question whether HE is a true syndrome.
Diagnosis is made in patients with an appropriate clinical picture and high antithyroperoxidase (anti-TPO) antibodies after infectious, toxic and metabolic causes of encephalopathy have been excluded (4).
However, objective data on the neurocognitive impairment in patients with HE and their improvement with treatment is limited. We present the case of a 28-year-old woman, illustrating a different approach to diagnosis and treatment of HE.
Case presentation
A 28-year-old woman was initially referred to the chronic fatigue clinic. She was a special needs teacher. During her teenage years she suffered from relapsing lethargy that coincided with menarche. She developed hypothyroidism secondary to autoimmune thyroiditis aged 20 years.
At the age of 26 years, she had been housebound for a year with fatigue. Her symptoms were dominated by lethargy, cognitive decline, poor concentration, generalised muscle aches and migraines.
She was unable to sustain employment. On examination, her body mass index (BMI) was 35.7 kg/m2, and she had no features of endocrinopathy.
etc
A novel assessment and treatment approach to patients with Hashimoto’s encephalopathy
Kate Laycock, Abhijit Chaudhuri, Charlotte Fuller, Zahra Khatami, Frederick Nkonge, and Nemanja Stojanovic
Barking, Havering and Redbridge University Hospitals NHS Trust, Queen's Hospital, Rom Valley Way, Romford, Essex, London, UK
Summary
Hashimoto’s encephalopathy (HE) is rarely reported with only a few hundred cases published. Diagnosis is made in patients with an appropriate clinical picture and high antithyroperoxidase (anti-TPO) antibodies after infectious, toxic and metabolic causes of encephalopathy have been excluded.
There is little objective data on the neurocognitive impairment in patients with HE and their improvement with treatment. We present the case of a 28-year-old woman with HE. Approach to management was novel as objective neuropsychological assessment was used to assess her clinical condition and response to treatment. Intravenous immunoglobulin (IVIg) as the first-line treatment instead of steroids. She responded well. The case illustrates that a different approach is required for the diagnosis and treatment of HE. A new diagnostic criteria is proposed that includes neurocognitive assessment, serum and CSF antibodies, an abnormal EEG and exclusion of other causes of encephalopathy. Furthermore, treatment should be tailored to the patient.
Learning points:
- Neurocognitive assessment should be carried out to assess the extent of brain involvement in suspected Hashimoto’s encephalopathy pre- and post- treatment.
- Treatment of Hashimoto’s encephalopathy should be tailored to the patient.
- Unifying diagnostic criteria for Hashimoto’s encephalopathy must be established.
Background
Hashimoto’s encephalopathy (HE) is characterised by a subacute, steroid-responsive encephalopathy in patients with positive antithyroid antibodies (1). The clinical presentation is highly variable; common clinical features include confusion, altered consciousness, tremor, transient aphasia, gait ataxia, seizures and sleep abnormalities. The natural history of the disease is relapsing-remitting (2). There have been just a few hundred cases reported in the literature. The average age of onset is 42 years, and it has an estimated prevalence of 2.1/100 000 people (3). The heterogeneity of the clinical presentation, the high prevalence of antithyroid antibodies in the general population and the lack of a clear underlying pathophysiological process has led many to question whether HE is a true syndrome.
Diagnosis is made in patients with an appropriate clinical picture and high antithyroperoxidase (anti-TPO) antibodies after infectious, toxic and metabolic causes of encephalopathy have been excluded (4).
However, objective data on the neurocognitive impairment in patients with HE and their improvement with treatment is limited. We present the case of a 28-year-old woman, illustrating a different approach to diagnosis and treatment of HE.
Case presentation
A 28-year-old woman was initially referred to the chronic fatigue clinic. She was a special needs teacher. During her teenage years she suffered from relapsing lethargy that coincided with menarche. She developed hypothyroidism secondary to autoimmune thyroiditis aged 20 years.
At the age of 26 years, she had been housebound for a year with fatigue. Her symptoms were dominated by lethargy, cognitive decline, poor concentration, generalised muscle aches and migraines.
She was unable to sustain employment. On examination, her body mass index (BMI) was 35.7 kg/m2, and she had no features of endocrinopathy.
etc