Category: Calcium/Bone Disorders

Monitor: 16

16 - PSEUDOHYPOPARATHYROIDISM WITHOUT ALLBRIGHT OSTEODYSTROPHY DEBUTING WITH POST ICTAL-PSICOSIS SEIZURES

Thursday, Apr 25
12:30 PM – 1:00 PM

Objective :

Chronic hypocalcemia is an unusual manifestation of disease, its causes are usually genetic with complications that can be life threatening. We report the case of a 25-year-old female patient known epilepsy since childhood treated with fenitoin 100 mg TID and cyanotic congenital cardiopathy treated with low dose aspirin, spironolactone 25 mg a day and furosemide 40 mg a day and 100 mcg a day of levothyroxine for hypothyroidism.
She presented with a six day history of multiple episodes of tonic-clonic generalized seizures. She was evaluated at a primary care facility and her antiepileptic medication dose was increased; she then developed psycomotor agitation with severe aggressiveness. She was admitted to the emergency service and found to have bilateral cataracts, nodular goiter, grade IV systolic murmur in the pulmonic and tricuspid foci, acrocyanosis and acropaquia. 


Methods : na


Results : na


Discussion :

Chovstek´s and Trousseau´s sign were found positive and her Glasgow was 12/15 (O4 V3 M5). Lab tests reported hemoglobin of 18g/dl, glucose 66 mg/dl, creatinine 1.57 mg/dl, creatine kinase 1831 U/L, Na:157meq/L and K:5.1 meq/L, with Ca: 5.7 mg/dl, albumin 4.2 g/dl, adjusted calcium 5.4 mg/dl and P: 10.7 mg/dl. A brain CT scan showed bilateral basal ganglia calcifications ; she was treated with calcium infusion.


In an endocrinology consult primary hypoparathyroidism was suspected due to the coexistence of cataracts and brain calcifications with hyperphospatemia. The association with congenital cyanotic cardiopathy supported the suspicion of Velocardial syndrome or Di George syndrome. She was treated with calcium infusion; after three days her adjusted calcium was 6.4 mg/dl, renal ultrasound was normal as were thyroid function and magnesium tests. Her Glasgow normalized even though her calcium continued to be 7. She was switched to oral calcium replacement and vitamin D3 which was found at 9 ng/dl and thiazide to increase renal calcium re absorbtion. An echocardiogram reported a 16-20 mm ventricular septal defect with tricuspid atresia, no overriding aorta and LVEF of 50%. PTH was  276 pg/ml with a confirmatory level of 240 pg/ml respectively; pseudo hypoparathyroidism was diagnosed; data associated with Allbright´s osteodystrophia was not found at physical or radiologic evaluation. She was released with an adjusted calcium at 8 mg/dl, she has been followed up, and is currently receiving oral calcium and vitamin D with no further problems at 8 months. 


Conclusion : Untreated chronic hypocalcemia can lead to brain calcifications and secondary epilepsy. We believe a more in depth study of her seizures would have resulted in earlier treatment and probable less neurological damage. 

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Maria Alejandra Ramos Guifarro

Chief of Endocrinology
Hospital Escuela Universitario
Tegucigalpa, Francisco Morazan, Honduras

Chief of Endocrinology Hospital Escuela

Miguel Osejo Betancourt

Internal Medicine
Hospital Escuela Universitario

.

Gerson Andino

Endocrinologist
Hospital Escuela Universitario

.

Daniel Andrés Guifarro Rivera

Medical Student
Universidad Nacional Autónoma de Honduras
Tegucigalpa, Francisco Morazan, Honduras

8th Year Medical Student

Carlos Rivera