A case of psychosis due to Fahr′s syndrome and response to behavioral disturbances with risperidone and oxcarbazepine

Abstract

Calcification of basal ganglia or Fahr's syndrome is a rare disease characterized by bilateral and symmetrical intracranial deposition of calcium mainly in cerebral basal ganglia. Motor and neuropsychiatric symptoms are prominent features. We report a case presented with a few motor symptoms, features of delirium and prominent psychiatric symptoms (disorganized behavior) predominantly evident after the improvement in delirium. Radiological findings were suggestive of bilateral basal ganglia calcification. Parathyroid hormone levels were low with no significant findings in other investigations and negative family history. Patient showed significant improvement in behavioral disturbances with risperidone, low dose of lorazepam, oxcarbazepine, and memantine.

Multiple calcifying pseudoneoplasms of the neuraxis (MCAPNON): Distinct entity, CAPNON variant, or old neurocysticercosis?

“…Serological autoimmune and inflammatory markers were negative. Further, the pattern of calcification was not compatible with that typically seen in metabolic disorders such as Fahr's disease (which usually affects the basal ganglia) 42 or hypoparathyroidism/pseudohypoparathyroidism. The pattern of the lesion also was inconsistent with phakomatoses, such as Sturge-Weber syndrome.…”

“…Their extensive, multifocal presence in the brain raises the possibility that they are precursors to the larger CAPNON‐like masses. Although there are known causes of vascular calcifications, including carbonic anhydrase II deficiency syndrome (marble brain syndrome), adult‐onset Fahr's disease, hypothyroidism, Sturge–Weber syndrome, Nasu‑Hakola disease, mitochondrial myopathy, encephalopathy, lactic acidosis and stroke‐like episodes (MELAS), myoclonic epilepsy with ragged red fibers (MERRF), and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), the patient's age, clinical, radiologic and histologic findings render unlikely these possibilities. Mineralizing microangiopathy can be seen with cancer treatment; however, the patient had no history of radiation or chemotherapy …”

Deconstructing Fahr's disease/syndrome of brain calcification in the era of new genes

“…The use of term "disease" for the "primary" or idiopathic calcification and "syndrome" to reflect the clinical/radiological picture, when a secondary cause is found, has been suggested. "Fahr's syndrome" and "Fahr's disease" have also been used interchangeably in some articles [7] and case reports [8,9]. Some authors ascribe the attribution of Fahr's name to this disorder a 'misnomer' [5].…”

Occurrence of Fahr's syndrome in patient suffering from systemic lupus erythematosus: A case report

“…Fahrov sindrom dijagnostikovaćemo kod mlađih pacijenata 30-40 godina, prisutnih simetričnih, bilateralnih intrakranijalnih kalcifikacija, utvrđene neke od endokrinopatija (idiopatski hipoparatireoidizam, sekundarni hipoparatireoidizam, pseudohipoparatireoidizam, hiperparatireoidizam, hipotireoidizam) ili prisustvo bolesti kao što su bruceloza, neuroferitinopatija, policistična lipomembranozna osteodisplazija, Sy Cockayne, Sy Aicardi-Gouteres, tuberozna skleroza, trovanje ugljen-monoksidom, neonatalna anoksija, trovanje olovom, posledice jonizujućeg zračenja, cerebralna hemoragija, encefalitis, celijakija, poremećaj vrednosti vitamina D, cisticerkoza, mitohondrijalna miopatija, lipoidna proteinoza itd. (2,7,18,19,20). Klinička slika i istorija primarne bolesti ukazuju na poreklo kalcifikata.…”