2016
DOI: 10.14744/nci.2015.47966
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Fahr’s Syndrome presenting with epileptic seizure, two case reports

Abstract: Fahr’s syndrome is a neuropsychiatric syndrome characterized by symmetrical and bilateral intracerebral calcifications located in the basal ganglia and usually associated with a phosphorus and calcium metabolism disorder. Clinical manifestations of Fahr’s syndrome vary; it may start at different ages and have a variety of presentations. This article discusses rare presentation of Fahr’s syndrome with epileptic seizure. These cases are important because they appear to be the first cases in the literature of Fah… Show more

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Cited by 4 publications
(5 citation statements)
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“…It can be seizures, pyramidal syndrome, akinetic hypertonic syndrome, cerebellar syndrome generally discreet, urinary problems, choreic athetosic movements, dysarthria or mumbling speech. However, seizures are rarely reported as revealing symptoms [10]. Cranial nerves involvement and bouts of benign intracranial hypertension are rare, while cases without neurological symptoms have been reported [1,7].…”
Section: Discussionmentioning
confidence: 99%
“…It can be seizures, pyramidal syndrome, akinetic hypertonic syndrome, cerebellar syndrome generally discreet, urinary problems, choreic athetosic movements, dysarthria or mumbling speech. However, seizures are rarely reported as revealing symptoms [10]. Cranial nerves involvement and bouts of benign intracranial hypertension are rare, while cases without neurological symptoms have been reported [1,7].…”
Section: Discussionmentioning
confidence: 99%
“…Concerning the etiopathogenic mechanism, the epileptic seizures may be caused by hypocalcemia due to primary hypoparathyroidism (explained by an increase in neuronal excitability induced by the drop in extracellular calcium concentration); however, another hypothesis is taken into consideration: the dysfunction of the cortico-basal connections and their interhemispheric relationships (13,14).…”
Section: Resultsmentioning
confidence: 99%
“…Fahr’s syndrome is often first diagnosed when CT imaging reveals bilateral intracerebral calcifications in the cerebral gray matter such as those seen in HY. However, the clinical presentations and age at the time of presentation vary greatly [ 3 ]. While basal ganglia calcifications are common idiopathic findings in older patients, it is more concerning in our patient given her younger age.…”
Section: Discussionmentioning
confidence: 99%
“…Although her EEG did not review any abnormalities, this study is limited by timing. Furthermore, seizures are rarely reported as the presenting symptoms of Fahr’s syndrome [ 3 ].…”
Section: Discussionmentioning
confidence: 99%