case 1 a 52-year - old male was examined in the clinic on first generalized tonic clonic seizure . blood tests were normal , except decreased levels of parathormone ( 6.46 pg / ml [ range : 15 - 65 pg / ml ] and calcium ( 5.7 mg / dl [ range : 8.6 - 10.2 mg / dl ] ) . he was not taking any medication , and there was no family history of dementia , movement disorder or other neurological illness . his brain computerized tomography ( ct ) scans demonstrated extensive , bilateral calcification of basal ganglia ( figure 1 ) . case 2 a 56-year - old male who had his first epileptic seizure , a generalized tonic clonic motor seizure , was referred to the hospital . blood tests were in normal range , except decreased vitamin d level ( 12 ug / l [ range : 20 - 120 ug / l ] ) . his ct scans demonstrated bilateral calcification of basal ganglia and cerebellum ( figure 2 ) . though it was patient s first seizure , sodium valproat was prescribed for 6 months in addition to vitamin d replacement treatment because of the eeg abnormality and no further seizure was seen after treatment . in the literature , varying manifestations of fahr s syndrome are described as memory disturbance , hallucination , delusions , personality change , and depression ; motor and phonic tics , stereotyped behaviors ; and extrapyramidal signs , such as parkinsonism and paroxysmal nonkinesigenic dyskinesia . hoque et al .   described a case of fahr s disease that presented with complex partial seizure and behavioral abnormalities . several families with basal ganglia calcification , representing a heterogeneous group of disorders with variable inheritance , have been described . , the cause of the intracranial calcification is abnormal calcium metabolism due to iatrogenic hypoparathyroidism . however , no phosphorus or calcium metabolism disorder , with the exception of decreased vitamin d level , was found in case 2 . since just decreased vitamin d level could not be the cause of intracerebral calcification , case 2 was diagnosed as primary fahr s syndrome . it was notable that no family history of neurological disease or psychiatric , demential or extrapiramidal signs were found in neurological examination of case 2 . it is possible that others with fahr s syndrome in this patient s family are asymptomatic . the cause of seizure in case 1 seems to be hypocalcaemia due to iatrogenic hypoparathyroidism . in addition , another theory of pathogenesis in these patients may be a dysfunction of cortico - basal connections and their interhemispheric relationship . in case 2 , fahr s disease  has been used to describe a characteristic pathological pattern of nonarteriosclerotic vascular calcification of the striopallidodentate system bilaterally , with variable deposition of ferro - calcareous concretions in cortical sulci , thalamus , cerebral white matter , and cerebellum . it is a misnomer , as fahr s original case was a rare example of hypoparathyroidism associated with calcification in the media of larger vessels in the cerebral white matter , without basal ganglia calcification . basal ganglia calcification may be categorized as idiopathic versus symptomatic ( especially of parathyroid insufficiency ) , or sporadic versus familial . prevalence of certain common neurological disorders , including dementia , stroke , and epilepsy , is similar in patients with incidentally discovered basal ganglia calcification and in age - matched controls . forty - two patients with incidentally discovered radiological basal ganglia calcification in the series of harrington et al . , and 33 cases reported by vles et al . murphy   found basal ganglia calcification in 53 of 7081 consecutive ct scans . in patients over 50 years of age , it was associated with clinical signs of basal ganglia dysfunction ( parkinsonism ) in only 3 patients . in a series of 42 cases of basal ganglia calcification revealed on ct ( performed for  various reasons  ) , puvanendran et al .   found a single patient with parkinsonism ( right - sided tremor and rigidity ) associated with dementia . most of reported families with basal ganglia calcification do not display clinical evidence of basal ganglia disease . the clinical feature of epileptic seizure in present cases was not typical for basal ganglia calcifications , whereas radiological appearances were those of fahr s syndrome . in conclusion , the present cases are important because it would appear that there is no case in the literature of fahr s disease presenting with generalized tonic clonic seizure . the seizures in such patients may be due to calcium metabolism abnormities and/or dysfunction of cortico - basal connections and their interhemispheric relationship .
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 fahr s syndrome presenting with generalized tonic clonic seizure .