Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis · 2026
Transient focal neurological episodes in hereditary transthyretin-related amyloidosis treated with liver transplantation: electroencephalographic insights and clinical features.
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Videira Gonçalo, Moura João, Freitas Joel, Alves Cristina, Neves Cardoso Márcio, Sousa Ana Paula, Pinto Miguel Mendonça, Pereira Diogo, Sousa Luísa, Cavaco Sara, Coelho Teresa
Аннотация
BACKGROUND: Hereditary transthyretin-related (ATTRv) amyloidosis may involve the central nervous system (CNS) years after liver transplantation, causing transient focal neurological episodes (TFNEs). Their mechanisms and electrophysiological correlates remain unclear.
METHODS: We retrospectively analyzed 155 patients with ATTRV30M (p.V50M) amyloidosis patients who underwent liver transplantation and had at least one electroencephalogram (EEG). TFNEs were categorized into five clinical subtypes. EEGs from outpatient and hospital settings were included.
RESULTS: TFNEs occurred in 76.8% of patients, most commonly as seizure-like (37.0%), transient ischemic attack-like (25.2%) or migraine-like (24.4%) episodes. Stroke-like TFNEs and bilateral tonic-clonic seizures were rarely the initial presentation (13.4%). Anti-seizure medications were prescribed to 77.3% of patients with TFNEs and 26.9% required two or more drugs. Focal slow EEG activity was detected in 40.0% of patients and was associated with TFNEs (OR = 2.91) and reduced survival (HR = 2.18). During acute TFNEs, focal slowing predominated, whereas epileptiform patterns were infrequent (19.5%). Cognitive impairment correlated with posterior dominant rhythm slowing and focal slow activity, particularly in language and executive domains.
CONCLUSIONS: TFNEs are common after liver transplantation in ATTRv amyloidosis. Focal slow EEG activity may indicate advanced CNS amyloid deposition, cognitive impairment and poorer prognosis.
METHODS: We retrospectively analyzed 155 patients with ATTRV30M (p.V50M) amyloidosis patients who underwent liver transplantation and had at least one electroencephalogram (EEG). TFNEs were categorized into five clinical subtypes. EEGs from outpatient and hospital settings were included.
RESULTS: TFNEs occurred in 76.8% of patients, most commonly as seizure-like (37.0%), transient ischemic attack-like (25.2%) or migraine-like (24.4%) episodes. Stroke-like TFNEs and bilateral tonic-clonic seizures were rarely the initial presentation (13.4%). Anti-seizure medications were prescribed to 77.3% of patients with TFNEs and 26.9% required two or more drugs. Focal slow EEG activity was detected in 40.0% of patients and was associated with TFNEs (OR = 2.91) and reduced survival (HR = 2.18). During acute TFNEs, focal slowing predominated, whereas epileptiform patterns were infrequent (19.5%). Cognitive impairment correlated with posterior dominant rhythm slowing and focal slow activity, particularly in language and executive domains.
CONCLUSIONS: TFNEs are common after liver transplantation in ATTRv amyloidosis. Focal slow EEG activity may indicate advanced CNS amyloid deposition, cognitive impairment and poorer prognosis.