Abstract
Hashimoto encephalopathy (HE) is associated with Hashimoto thyroiditis. Clinically
it presents with variable symptoms like seizures, neuropsychiatric changes or focal
neurological deficits. Autoimmune phenomena are hypothesized for the pathogenesis.
HE has mainly been described in the adult population. We present two 14-year-old patients
who presented with recurrent seizures and mental decline. SPECT and PET scans showed
distinctly pathological changes. Both patients were diagnosed with HE and improved
dramatically on steroids. We feel that HE is a rare but important differential diagnosis
of encephalopathy also in the pediatric population. As this disease responds well
to steroids, we recommend to obtain basic thyroid function tests as well as thyroid
antibodies in all cases of unexplained encephalopathy or unexplained status epilepticus.
Key words
Hashimoto Encephalopathy - Children - Hashimoto Thyroiditis - Status Epilepticus -
Thyroid Antibodies
References
- 1
Balestri P, Grosso S, Garibaldi G.
Alternating hemiplegia of childhood or Hashimoto's encephalopathy?.
J Neurol Neurosurg Psych.
1999;
66
548-549
- 2
Brain L, Jellinek E H.
Hashimoto disease and encephalopathy.
Lancet.
1966;
2
512-514
- 3
Byrne O C, Zuberi S M, Madigan C A, King M D.
Hashimoto's thyroiditis - a rare but treatable cause of encephalopathy in children.
Eur J Paediatr Neurol.
2000;
4
279-282
- 4
Dayan C M, Daniels G H.
Chronic autoimmune thyroiditis.
N Engl J Med.
1996;
335
99-107
- 5
Forchetti C M, Katsamakis G, Garron D C.
Autoimmune thyroiditis and a rapidly progressive dementia: Global hypoperfusion on
SPECT scanning suggests a possible mechanism.
Neurology.
1997;
49
623-626
- 6
Gucuyener K, Serdaroglu A, Bideci A, Yazman Y, Soysal A S, Cinaz P J.
Tremor and myoclonus heralding Hashimoto's encephalopathy.
J Pediatr Endocrinol Metab.
2000;
13
1137-1141
- 7
Hernandez Echebarria L E, Saiz A, Graus F, Tejada J, Garcia J M, Clavera B, Fernandez F.
Detection of 14-3-3 protein in the CSF of a patient with Hashimoto's encephalopathy.
Neurology.
2000;
54
1539-1540
- 8
Kothbauer-Margreiter I, Sturzenegger M, Komor J, Baumgartner R, Hess C W.
Encephalopathy associated with Hashimoto thyreoiditis: Diagnosis and treatment.
J Neurol.
1996;
243
585-593
- 9
McCabe D JH, Burke T, Connolly S, Hutchinson M.
Amnesic syndrome with bilateral mesial temporal lobe involvement in Hashimoto's encephalopathy.
Neurology.
2000;
54
737-739
- 10
Querol Pascual M R, Aguirre Sanchez J J, Velicia Mata M R, Gahete Jimenez C, Duran
Herrera M C, Gonzalez Dorrego F.
Hashimoto's encephalitis: a new case with spontaneous remission.
Neurologia.
2000;
15
313-316
- 11
Rallison M L, Dobyns B M, Keating F R, Rall J E, Tyler F H.
Occurrence and natural history of chronic lymphocytic thyroiditis in childhood.
J Pediatr.
1975;
86
675-682
- 12
Seipelt M, Zerr I, Nau R, Mollenhauer B, Kropp S, Steinhoff B J. et al .
Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease.
Neurol Neurosurg Psych.
1999;
66
172-176
- 13
Shaw P J, Walls T J, Newman P K, Cleland P G, Cartlidge N EF.
Hashimoto encephalopathy: a steroid-responsive disorder associated with high anti-thyroid
antibody titers, report of 5 cases.
Neurology.
1991;
41
228-233
- 14
Shein M, Apter A, Dickerman Z, Tyano S, Gadoth N.
Encephalopathy in compensated Hashimoto thyroiditis: a clinical expression of autoimmune
cerebral vasculitis.
Brain Dev.
1986;
8
60-64
- 15
Sybesma C A, v Pinxteren-Nagler E, Sinnige L G, van Oostrom J C, de Vries T W.
Hashimoto encephalopathy in a 12-year-old girl.
Eur J Pediatr.
1999;
158
867-868
- 16
Takahashi S, Mitamura R, Itoh Y, Suzuki N, Okuno A.
Hashimoto encephalopathy: etiologic considerations.
Pediatr Neurol.
1994;
11
328-331
- 17
Vasconcellos E, Pina-Garza J, Fakhoury T, Fenichel G.
Pediatric manifestations of Hashimoto's encephalopathy.
Pediatr Neuro.
1999;
20
394-398
- 18
Watemberg N, Willis D, Pellock J M.
Encephalopathy as the presenting symptom of Hashimoto's thyroiditis.
J Child Neurol.
2000;
15
66-69
Prof. Dr. R. Korinthenberg
Department of Neuropediatrics, University Children's Hospital
Mathildenstr. 1
79106 Freiburg
Germany
Email: rudokori@kikli.ukl.uni-freiburg.de