Electroencephalogram (EEG) showed paroxysmal generalized sharp waves. Various anticonvulsant and antipsychotic medications were prescribed for the patient after he developed myoclonic jerk movements and other mental disorders. plasmaphresis was started, to which the patient showed dramatic response. strong class=”kwd-title” Keywords: Hashimoto encephalopathy, Epilepsy, Iran Introduction Hashimoto Encephalopathy (HE) is defined as a steroid responsive encephalopathy associated with autoimmune thyroiditis (1). Symptoms are non-specific and include a wide spectrum of neurologic deficits. Among all neurologic symptoms, status epilepticus is a rare presentation (2). Herein, we report a case of HE with an unusual presentation of drug resistant status epilepticus. Case Report A 33 yr old man, previously diagnosed with hypothyroidism, presented with decreased level of consciousness and generalized tonic-clonic (GTC) seizure to Namazi hospital, Shiraz, Iran, during April, 2015. The seizure lasted for about 1C2 Rabbit polyclonal to ERO1L min and the patient recovered completely after that episode, so the patient did not refer for any medical care. After a few days, the patient experienced another seizure-like attack and developed urinary and defecation incontinency, aphasia, total body weakness and disorientation. At that time, the patient was admitted to a hospital and in 7 days of his admission, his signs and symptoms improved and he was discharged with phenytoin, carbamazepine and levothyroxine. After these events, the patient had three more hospital admissions due to similar symptoms. After a one-month symptom free period, he experienced another GTC seizure attack. The patient experienced four episodes of GTC seizures during the 24 h prior to his last admission, during which the patient did not regain consciousness after the second episode of GTC seizure. On admission, the patient was aphasic, did not obey completely and had impaired gait. The GTC seizures were controlled by administering intra venous phenytoin. During the hospital admission, the patient developed myoclonic jerks, followed by mental disorders including aphasia, ataxia, loss of verbal communication, eye contact and complete loss of obedience. The patient also developed urinary tract infection for which he received ceftriaxone and was resolved. His laboratory tests for viral markers including the human immunodeficiency and hepatitis C viral antibodies and hepatitis B virus antigen were all negative. The Wright plus test was negative. The Venereal Disease Research Laboratory test (VDRL) was negative. His serum Anti-thyroid peroxidase antibody (Anti-TPO Ab) and blood ammonia levels were high (1095.8 IU/ml and 189.3 mol/L, respectively). His CPK, lactate dehydrogenase (LDH) and CRP levels were 137 mg/L, 437 U/L and 58 mg/l, respectively. Although the CRP level decreased (28 mg/l) after receiving ceftriaxone. Thyroid Lucidin function tests (TFT) showed low levels of T3 and T4 Lucidin with high levels of thyroid stimulating hormone (TSH). Other blood tests, including complete blood count, liver function test, kidney function tests and electrolytes, erythrocyte sedimentation rate, homocysteine levels and the peripheral blood smear were all normal. Brain magnetic resonance Imaging (MRI) and brain computed tomography (CT) scan were normal. Electroencephalogram (EEG) showed paroxysmal generalized sharp waves. Various anticonvulsant and antipsychotic medications were prescribed for the patient after he developed myoclonic jerk movements and other mental disorders. The treatment included a combination of sodium valproate, clonazepam and levetiracetam. The combined treatment controlled the myoclonic jerks but did not improve the mental problems. With no significant Lucidin improvement in his condition, he underwent two lumbar punctures (LP). The first LP was to rule out herpes encephalopathy and the second was done to check the possibility of HE. The first Cerebrospinal Fluid (CSF) analysis showed an elevated cell count (19500/mm3) with two WBCs (two segments), elevated protein levels (56 mg/dL) and normal Glucose and LDH levels. The second CSF analysis had a total cell count of 1600/mm3 with a total WBC count of 10 (five lymphocytes, 5 segments) and a high titer of Anti-TPO antibodies. With high suspicion of HE, treatment was started. As the first line of treatment, pulsed methyl prednisolone (10 mg) was administered, however the patient showed little improvement. Therefore, plasmaphresis was started, to which the patient showed dramatic response. Discussion With a prevalence of 2.1 in 100000 (3), HE is considered a rare condition. Due to its vague clinical manifestations (2) and uncertain diagnostic tests, it is mostly diagnosed by excluding other conditions (3). The diagnosis is made when high anti-thyroid antibodies accompany encephalopathy symptoms (4), and no other infectious, metabolic or paraneoplastic processes are responsible for a patients condition (3). Clinical Manifestations Patients clinical symptoms can be categorized into two.