Paediatria Croatica, Vol. 58 No. 4, 2014.
Pregledni rad
https://doi.org/10.13112/PC.2014.47
Autoimmune encephalopathies in children: clasifi cation, diagnosis and treatment
Nina Barišić
; Klinički bolnički centar Zagreb
Nina Vrsaljko
Vanja Zvonar
Goran Tešović
Sažetak
Autoimmune encephalopathies are clinically manifested as limbic or diff use encephalitis. According to the etiology, they are classifi
ed as paraneoplastic and non-paraneoplastic. Signs and symptoms of autoimmune encephalitis are variable. The symptomatology
commonly includes headache and epileptic attacks often progressing to epileptic status, movement disorders (ataxia, dyskinesias,
chorea, dystonia and tremor), behavior changes, cognitive impairments, psychoses and various degrees of disorders of
consciousness. Faciobrachial dystonic seizures can precede the development of limbic encephalitis. Autonomic dysfunction, sleep
disorders and hypoventilation are often present. The development of autoimmune encephalopathies can be induced by either
tumor or viral antigens. However, in a signifi cant number of cases, disease triggers remain unidentifi ed. According to the localization
of target antigens, autoimmune encephalopathies can be divided into those caused by antibodies against intracellular antigens
(Ma2,Hu) and those caused by antibodies against cell surface antigens, i.e. synaptic antigens (N-methyl-D-aspartate receptor
(NMDAR), voltage-gated potassium channel complex/LGI1). The former are paraneoplastic in origin, more often in adults and
respond poorly to immunotherapy. The latter ones can aff ect children as well, and are usually-responsive to immunotherapy. Antibodies
can be detected in both cerebrospinal fl uid and serum. Mild pleocytosis and/or oligoclonal bands can be found in cerebrospinal
fl uid but in some patients the cerebrospinal examination fi ndings can be completely normal. The electroencephalography fi nding
consists of diff use, slow dysrhythmic encephalopathic changes or so-called extreme delta brushes and focal epileptogenic
changes, i.e. paroxysmal bursts in case of limbic encephalitis. Magnetic resonance image fi nding of the brain is usually normal or
presents transient sub/cortical hyperintensities in T2-weighted images but is signifi cant for the diagnosis of limbic encephalitis.
Early recognition of autoimmune encephalopathy is of utmost importance because of the need of proper diagnostic procedure and
timely introduction of appropriate therapy.
Ključne riječi
encephalopathies; autoimmune encephalitis; electroencephalogram; NMDA receptor; synaptic antigens, pediatric
Hrčak ID:
142338
URI
Datum izdavanja:
23.12.2014.
Posjeta: 6.413 *