Response to treatment with corticoids in a case of inflammatory amyloid angiopathy without performing a biopsy
*Correspondencia: Dra. Patricia de la Riva Juez. Servicio de Neurología. Hospital Donostia. P.º Doctor Beguiristain, 109. E-20014 San Sebastián (Guipúzcoa).
Fax: +34 944 007 109.
E-mail: patricia.delarivajuez@gmail.com
Introduction: Inflammatory amyloid angiopathy (IAA) is an infrequent presenting symptom of the recently recognised cerebral amyloid angiopathy and its definitive diagnosis is reached by means of pathological analyses.
Aim: We report the case of a male patient with IAA and good clinical, neuropsychological and neuroimaging response to treatment with corticoids; a biopsy of brain tissue was not considered necessary.
Case report: The patient, 68 years old and diagnosed with Alzheimer's disease, suffered from generalised seizures followed by a language disorder and hemiparesis of the right-hand side. A magnetic resonance imaging scan showed a lesion displaying infiltrating behaviour in the left hemisphere and multiple instances of microbleeding. Clinical and radiological features suggested IAA and treatment was established with corticoids. Neuroimaging and neuropsychological tests revealed a notable improvement at 30 days after beginning treatment with immunosuppressants. The genotype was ApoE e4/e4. The need to perform a biopsy of brain tissue was ruled out.
Conclusions: The case described here suggests that, in individualised cases with clinical and radiological features that are characteristic of IAA, it may be possible to establish an empirical treatment with corticoids with a probability diagnosis and perform a biopsy of brain tissue in the event of a lack of response to treatment.
Objetivo Se presenta un paciente con AAI con buena respuesta clínica, neuropsicológica y de neuroimagen al tratamiento con corticoides y en el que no se consideró necesario practicar biopsia cerebral.
Caso clínico Varón de 68 años con diagnóstico de enfermedad de Alzheimer que sufrió una crisis convulsiva generalizada seguida de trastorno del lenguaje y hemiparesia derecha. La resonancia magnética mostró una lesión de comportamiento infiltrante hemisférica izquierda y múltiples microsangrados. La clínica y radiología fueron sugestivas de AAI y se instauró tratamiento corticoideo. La neuroimagen y los tests neuropsicológicos mostraron una notable mejoría a los 30 días del inicio del tratamiento inmunosupresor. El genotipo fue ApoE epsilon4/epsilon4. Se desestimó la realización de biopsia cerebral.
Conclusiones El caso descrito sugiere la posibilidad de, en casos individualizados con clínica y radiología características de AAI, instaurar tratamiento empírico con corticoides con diagnóstico de probabilidad y realizar biopsia cerebral en caso de que no haya respuesta al tratamiento.
