Two Studies about Hashimoto encephalopathy

1-Hashimoto’s encephalopathy: how different it could be?

Joana Ramalho, Mauricio Castillo

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 Hashimoto’s encephalopathy is a rare neurological complication of autoimmune thyroid disease characterized by acute or subacute onset of cognitive impairment, psychiatric disorders, seizures, myoclonus and stroke-like symptoms. 

It is a poorly understood and often misdiagnosed entity also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) or nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM).

The diagnosis is challenging since the underlying thyroid disease is often subclinical and symptoms mimic other neurological conditions. 

Neuroimaging findings are frequently not helpful and brain magnetic resonance imaging (MRI) is often normal. Almost all reported cases of Hashimoto’s encephalopathy have been responsive to high doses of corticosteroids.

We report a case of Hashimoto’s encephalopathy with atypical and partially reversible MRI findings. T2 weighted MRI images revealed bilateral symmetric areas of increased signal in the mesial temporal lobes and basal ganglia. 

Despite clinical and imaging improvement after steroid therapy some memory deficits and MRI abnormalities persisted.

Fuente:  http://radiology.casereports.net/index.php/rcr/article/viewArticle/445/

2- Hashimoto encephalopathy presenting as progressive myoclonus epilepsy syndrome 

  
European Journal of Paediatric Neurology, Available online 25 July 2012
Ravindra Arya, Vidhu Anand, Maya Chansoria

We report an 11 year old girl with multi-focal spontaneous myoclonus, generalized seizures and behavioural changes. 

She was found to have sub-clinical hypothyroidism and elevated anti-thyroid peroxidase antibodies. 

A diagnosis of Hashimoto encephalopathy (or Steroid Responsive Encephalopathy with Autoimmune Thyroiditis) was made in view of consistent clinical and laboratory features. 

The patient showed and maintained significant improvement with valproate, clonazepam and long term prednisolone. 

Other findings included mild cerebral cortical grey matter atrophy on brain magnetic resonance imaging and intermittent slowing with generalized poly-spike-and-wave discharges on electroencephalography. 

Other causes of progressive myoclonus epilepsy (PME) syndrome including:

1.  subacute sclerosing panencephalitis, 
2. Lafora body disease, 
3. Juvenile Neuronal Ceroid Lipofuscinosis,
4. Late onset gangliosidosis, 
5. Sialidosis, mitochondrial disorders and 
6. coeliac disease 

were ruled out by appropriate investigations. 

No other auto-immune abnormality was detected.

 This case emphasizes that Hashimoto encephalopathy should be considered in the etiologies of PME syndrome in adolescents since it is a treatable entity.

Keywords

• Hashimoto encephalopathy;
• Steroid responsive encephalopathy with autoimmune thyroiditis;
• Progressive myoclonus epilepsy syndrome;
• Anti-thyroid antibodies

Case studio

Authors

• Ravindra Arya^a,
• Vidhu Anand^b,
• Maya Chansoria^{c, ,}

• ^a Comprehensive Epilepsy Center, Division of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
• ^b Post Graduate Institute of Medical Sciences, Rohtak, India
• ^c Department of Pediatrics, N.S.C.B. Medical College, Garha, Jabalpur 482 003, MP, India

•  Available online 25 July 2012.
•  Corresponding author. Tel.: +91 761 242 2117x296; fax: +91 761 242 2119.

• Fuente: European Journal of Paediatric Neurology,
• http://dx.doi.org/10.1016/j.ejpn.2012.07.001,