An unusual cause of cerebellovestibular symptoms

Abstract

Hashimoto encephalopathy (HE) is a controversial autoimmune disorder, probably underdiagnosed, that causes a wide variety of neurological manifestations. Symptoms differ among patients and may be very severe in some cases. However, it can be treated, with a very good prognosis. In our case, a teenaged girl with a family history of migraine, vitiligo and benign paroxysmal positional vertigo presented with severe ataxia, vomiting and hypotension. She had a history of similar, but milder, symptoms and was misdiagnosed several times. She had subclinical hypothyroidism, and high levels of antithyroid antibodies. There were abnormal MRI and visual evoked potential findings. After excluding other more common causes, we diagnosed her as having ‘Hashimoto Encephalopathy’, and started treatment with corticosteroids, on which she showed dramatic improvement. After about 2 years of presentation, the patient is able to continue her life independently.

Clinical characteristics of paediatric Hashimoto's encephalopathy

“…Epileptic features varied from generalized tonic-clonic convulsions with duration of less than 5 min to epileptic state with duration of several hours. Other frequently reported neurologic symptoms were coma (18), dysarthria (13), hemiparesis (9), and ataxia (11). Hallucinations (21), confusion (17), anxiety (4), and depression (3) were reported psychiatric symptoms.…”

Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) Presenting with Pure Cerebellar Ataxia

“…Uncommon movement disorder phenomenologies in SREAT include ataxia without encephalopathy, [13][14][15][16][17][18][19] chorea, [20][21][22] and dystonia. 22 Here, we report two patients who presented with subacute onset of pure or predominant cerebellar ataxia and negative autoimmune and paraneoplastic workups, except anti-thyroid antibodies.…”