pediatric idiopathic hypoparathyroidism with extensive intracranial calcifications outside the basal ganglia ( bg ) is extremely rare with less than 10 cases worldwide . an 11-year - old saudi male child presented with tetany with otherwise normal neurological and other body system examination diagnoses severe hypocalcemia for differential diagnosis . he had normal hemoglobin , electroencephalogram , and skeletal survey , with negative autoantibodies to alpha and omega interferons and negative genetic testing for glial cell missing 2 ( gcm2 ) and calcium - sensing receptors ( casrs ) excluding known causes of hypoparathyroidism . this case presents a rare entity of idiopathic hypoparathyroidism with extensive intracranial calcification , not only in bg but also outside the extrapyramidal system with normal mentality , development , pubertal achievement , and neurological examination . to our knowledge idiopathic hypoparathyroidism is a diagnosis of exclusion after ruling out all known causes of hypoparathyroidism . it is associated with bg calcifications , but extensive intracranial calcifications outside the bg are extremely rare . hypoparathyroidism is an endocrine disorder caused as a result of congenital disorders , iatrogenic causes , infiltration of the parathyroid glands , suppression of parathyroid function , or idiopathic mechanisms . idiopathic hypoparathyroidism is an uncommon condition of unknown etiology diagnosed by exclusion of different possible etiologies . the most common site is often globus pallidus , but extensive intracranial calcification beyond the extrapyramidal system is rare . we present case of idiopathic hypoparathyroidism with extensive intracranial calcification , without features of extrapyramidal system symptom or previous history of neurological manifestation , and presented with tetany . an 11-year - old saudi male child with negative past medical history for seizures , abnormal movements , hematological or other chronic diseases , hemochromatosis , radiation or other abnormal environmental exposures , and no history of previous thyroid surgery or other surgeries presented to the pediatric emergency department with sustained tetanic contraction of both hands for 2  hours with positive chvostek and trousseau signs . meticulous history revealed history of tingling and numbness sensations and sometimes facial twitches used to occur with exercise or hyperventilation started few years ; roughly 4 years as the child remembers ; before current presentation . further examination revealed average anthropometric measurements ; lying on the 50th centile ; with good nutritional status . the patient was fully conscious , cooperative , well oriented to time , person and place , with average mood , memory , and intelligence along with normal gait and speech . he had no signs suggestive of cerebellar , extrapyramidal , or any other neurological affection . no skin or mucus membrane abnormalities were noted , including normal hair with no hair loss , no vitiligo , or mucucutaneous candidiasis . the patient was immediately admitted to the pediatric intensive care unit and connected to cardiac monitor . his initial investigations before calcium supplementation revealed severe hypocalcaemia ( 0.6  mol / l ) and hyperphosphatemia with normal magnesium level . one - alpha - hydroxy - cholecalciferol was then started . the patient condition improved within 24  hours and the patient was transferred to the pediatric ward . ophthalmological assessment including slit lamp and fundus examinations was normal with no cataract and no any fundus abnormalities . no evidence of heterotrophic calcium deposition in the skin , no brachydactylies , and no evidence of spondyloarthropathy was noted . further investigations looking for the cause of severe hypocalcemia revealed undetectable serum parathyroid hormone ( pth ) with normal blood picture , erythrocyte sedimentation rate , c - reactive protein , serum glucose level , serum total proteins , serum albumin , liver functions , renal functions , thyroid functions , serum iron - ferritin - total iron binding capacity , morning adrenocorticotropin , and cortisol tests . no signs suggestive of other autoimmune diseases with negative anti - alpha and anti - omega interferons antibodies excluding autoimmune polyglandular syndrome i were noted . anti - parietal cell , anti - liver - kidney - microsomal , and anti - tissue - transglutaminase antibodies were all negative . skeletal survey of the patient was normal with no calvarial thickening , no soft tissue calcification , no premature closure of epiphysis , and no osteosclerosis ; computed tomography ( ct ) of brain showed bilateral extensive nonenhancing - hyperdense lesions ( calcifications ) involving the thalamus , dentate nuclei , putamen , globus pallidus , caudate nucleus , and subcortical white matter with hu of 40 to 70 ( fig . 1 ) . ct brain showing dense calcification in the head of caudate nucleus , basal ganglia , and the subcortical white matter region bilaterally in the cerebral cortex . genetic testing for glial cell missing 2 ( gcm2 ) and casr genetic mutations was sent for the patient and came out to be negative . on the basis of the clinical , hormonal , radiological , and genetic findings , known causes of hypoparathyroidism were excluded . the patient was discharged on regular oral calcium gluconate 2  g / day and one - alpha - hydroxy - vitamin - d3 of 1  g / day with regular follow - up to adjust doses based on serum calcium and phosphorus levels . the patient is being followed up for 5 years now with good serum calcium levels with no recurrence of tetany , and frequent adjustments of medication doses were required . the patient is growing well , achieving normal development without any neurological sequels with normal pubertal development and intelligence . follow - up ct brain revealed increased density of the calcifications ( hu of 160 ) . skeletal survey and we report this case after approval of the research and ethical committees of alhada armed forces hospital , taif , ksa . written informed consent was signed by the father for reporting and publishing the patient 's clinical data . pediatric idiopathic hypoparathyroidism has been associated with bg calcifications in a considerable number of patients , but extensive intracranial calcifications outside the bg are extremely rare with only few reports in pediatrics ( table 1 ) . although most of bg calcifications are a - symptomatic , but this patient is unique in having no neurological affection with normal intelligence in spite of the extensive bilateral cerebral cortex and intracranial calcifications added to the bg calcification . . clinical characteristics of reported pediatric patients with idiopathic hypoparathyroidism who have extensive intracranial calcifications . we did not include the report of soffer et al in table 1 , as no intracranial calcifications were detected in their patients with idiopathic hypothyroidism and extrapyramidal manifestations . in addition , anti - alpha and anti - omega interferons antibodies that have a high sensitivity and specificity in detecting autoimmune polyglandular syndrome i were negative ; also , anti - casr was negative , excluding autoimmune hypoparathyroidism . our report is the first to study both casr and gcm2 genetic mutations as compared with the previous 5 reports that made the diagnosis of idiopathic hypoparthyroidism in our patient on solid basis . multidisciplinary long - term follow - up by endocrinologist , neurologist , mental health consultant , nephrologist , and general pediatrician is planned to achieve excellent care of the patient and for better characterization of disease progression .
abstractrationale : pediatric idiopathic hypoparathyroidism with extensive intracranial calcifications outside the basal ganglia ( bg ) is extremely rare with less than 10 cases worldwide.patient concerns : an 11-year - old saudi male child presented with tetany with otherwise normal neurological and other body system examination diagnoses severe hypocalcemia for differential diagnosis.interventions:further investigations revealed hyperphosphatemia and undetectable serum intact parathyroid hormone . 
 brain computed tomography revealed bg and extensive brain calcifications . 
 he has no dysmorphic features , vitiligo , mucocuataneous manifestations , or hair loss . 
 he had normal hemoglobin , electroencephalogram , and skeletal survey , with negative autoantibodies to alpha and omega interferons and negative genetic testing for glial cell missing 2 ( gcm2 ) and calcium - sensing receptors ( casrs ) excluding known causes of hypoparathyroidism.outcomes:this case presents a rare entity of idiopathic hypoparathyroidism with extensive intracranial calcification , not only in bg but also outside the extrapyramidal system with normal mentality , development , pubertal achievement , and neurological examination . to our knowledge 
 , this is the first report from saudi arabia in pediatrics.lessons:idiopathic hypoparathyroidism is a diagnosis of exclusion after ruling out all known causes of hypoparathyroidism . 
 it is associated with bg calcifications , but extensive intracranial calcifications outside the bg are extremely rare .