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Idiopathic Hypoparathyroidism in a Blind, Deaf, Elderly Woman with Dementia

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Idiopathic Hypoparathyroidism in a Blind, Deaf, Elderly Woman with Dementia Idiopathic hypoparathyroidism in a blind, deaf, elderly woman with dementia JUDITH A. ABERG, MD, AND MARTIN J. GORBIEN, MD EMENTIA among elderly persons is a diagnostic surveillance), and likelihood of multiple growing sociomedical problem. An esti- illnesses, some of which may be inapparent or may mated 10% to 15% of persons older than present atypically. age 65 may be cognitively impaired, as Idiopathic hypoparathyroidism is a rare metabo- D 1 may up to 76% of all patients in nursing homes. In lic disorder primarily affecting children and young addition, in one study, dementia was found to have adults. Characterized by seizures, tetany, and cata- been misdiagnosed in 30% to 50% of cases when racts, it can be readily reversed by correcting the complete follow-up was obtained 5 to 10 years after serum calcium level.3"9 We present a case of multi- the initial evaluation. The four most common factorial dementia and idiopathic hypoparathyroid- causes of reversible dementia are medications, de- ism in an elderly woman. pression, metabolic disorders, and central nervous system lesions.1,2 CASE HISTORY Alzheimer's disease, the most common cause of progressive dementia, remains a clinical diagnosis of A 74-year-old blind, deaf, and mute white woman exclusion, and other, potentially correctable causes presented for treatment of "visual hallucinations" should always be sought and ruled out.2 In addition, and altered mental status. Her blindness had been the presumed cause of the dementia must be reas- caused by glaucoma, and both lenses had been surgi- sessed periodically to detect any occult superim- cally removed because of dense cataracts. She had posed illness. Elderly demented persons are at con- been deaf since an early age as the result of a pertussis siderable risk of undetected but treatable illness as a infection. She was able to communicate by signing result of what Larson1 has called "triple jeopardy": and feeling other people's signs with her hands. inability to provide an accurate history, social isola- The patient's family had noted changes in her tion (which may effectively remove a person from mental status and episodes of emesis and urinary retention over the past year. She had been admitted to another hospital approximately 10 months pre- From the Department of Infectious Disease, Washington viously because of ileus, electrolyte imbalance, renal University School of Medicine (J.A. A.), St. Louis, and the insufficiency with acute urinary retention, anemia, Section of General Internal Medicine, The University of Chi- and acute delusional disorder. The delusional disor- cago Pritzker School of Medicine (M.J.G.), Chicago. der resolved approximately 4 months later; sub- Address reprint requests to M.J.G., Section of General Internal Medicine, The University of Chicago Pritzker School sequently the episodes of confusion began to recur. of Medicine, 5841 South Maryland Avenue, MC 3051, Chi- Laboratory evaluations at the other hospital re- cago, IL 60637. vealed low serum concentrations of calcium and 156 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 63 • NUMBER 3 Downloaded from www.ccjm.org on October 2, 2021. For personal use only. All other uses require permission. IDIOPATHIC HYPOPARATHYROIDISM • ABERG AND GORBIEN mid-molecule parathyroid hormone. The patient TABLE had been given vitamin D and calcium carbonate RESULTS OF LABORATORY TESTS ON ADMISSION supplements (500 mg of calcium four times a day). However, compliance had been an issue, and her Test Result children were not sure how much she was actually Hemoglobin 10.2 g/dL taking. Hematocrit 31 % Beginning several weeks before this hospital ad- Calcium 7.9 mg/dL mission, the family would find the patient signing to Phosphorus 4.4 mg/dL herself, as if in a conversation. When asked, she Magnesium 2.0 mg/dL would sign that she thought someone was there. In Alkaline phosphatase 81 IU/L addition, her daughter described "bizarre seizures" in 25-Hydroxyvitamin D3 46 ng/mL which the patient would extend all four extremities 1,25-Dihydroxyvltamln D3 29 pg/mL and jerk. This was followed by spontaneous, invol- Parathyroid hormone (intact) <4 pg/mL untary urination. The patient would begin to sign Urinary cyclic 0.7 |imole/g of adenosine monophosphate creatinine again approximately 2 minutes after the episode, but (cAMP) it was not clear whether she actually lost conscious- Blood urea nitrogen (BUN) 61 mg/dL ness. The family also reported that the patient had Creatinine 4.6 mg/dL daily spells of disorientation to time and place. She Vitamin B12 875 pg/mL had been to a local emergency room several times Folate 7.3 ng/mL because of these episodes, but no clear cause for Iron 37 ng/dL them was found. Total iron-binding capacity 168 ng/dL The patient had a history of hypothyroidism (for Percent iron saturation 22 % which she received thyroid hormone replacement Ferritin 180 ng/mL therapy), hypertension, arrhythmias, and seizures Thyroid-stimulating hormone 12.3 U/mL due to electrolyte imbalances. She had undergone Free thyroxine (T4U) 0.86 ng/dL multiple extractions of calcium deposits in all fin- Free thyroxine index 10.2 % gers and toes, an implantation of a prosthetic joint Triiodothyronine (T3) 65 ng/dL in the second finger of her left hand, and a pace- maker insertion because of a Mobitz type II heart block. shown in the Table. Results of a Cortisol and cosyn- Physical and laboratory examination tropin stimulation test were normal. The electroen- The patient had severe orthostatic hypotension: cephalogram was unremarkable. A computed to- her blood pressure was 130/70 mm Hg supine but mographic scan of the head revealed coarse diffuse only 60 mm Hg (palpated, systolic) standing. The intraparenchymal calcifications within the basal skin was unremarkable. Her pupils were irregular ganglia, thalamus, both cerebellar hemispheres, and and did not react to light. The tympanic membranes the gray-white junction (Figure). Computed to- had moderate scarring. The thyroid was not en- mography of the orbits confirmed the absence of larged, and the lungs were clear. Cardiac examina- both lenses. The electrocardiogram revealed a pro- tion revealed normal heart sounds with occasional longed Q-T interval. ectopic beats and a grade 1 of 6 systolic murmur. The abdominal examination was unremarkable. Hospital course The patient was oriented to time but not to place. On the first day, we started fluid resuscitation Cranial nerves V and VII through XII were intact. (dextrose 5% and saline 0.45% at 100 mL/hour) and The extraocular muscles were difficult to evaluate calcium supplementation (calcium carbonate 4 g by but appeared intact. Torsional nystagmus was noted mouth daily). Given her history of congestive heart on fixed gaze. The deep tendon reflexes were sym- failure, she was not given any normal-saline boluses, metric and reduced in the lower extremities. Babin- which could potentially drive down her calcium ski's reflex was absent. level even more. Nevertheless, by the fourth day her Her serum calcium concentration on admission calcium level had further decreased to 6.4 mg/dL. was 7.9 mg/dL; other initial laboratory results are We therefore gave two ampules of calcium glucon- MAY . JUNE 1996 CLEVELAND CLINIC JOURNAL OF MEDICINE 157 Downloaded from www.ccjm.org on October 2, 2021. For personal use only. All other uses require permission. IDIOPATHIC HYPOPARATHYROIDISM • ABERG AND GORBIEN longer exhibited signs of dementia, disorientation, or the seizures described above. Because she was doing well and was able to continue living with her family, her care was transferred to a local physician. DISCUSSION Hypoparathyroidism most often occurs as a complication of thyroid or parathyroid surgery. Idiopathic and pseudohypoparathyroidism (Al- bright hereditary osteodystrophy) are both rare. In all three forms, blood levels of calcium are low (< 8.5 mg/dL) and phosphorus levels are high (> 4-5 mg/dL). In contrast, parathyroid hormone (PTH) levels are low (< 10 pg/mL) in surgical and idi- opathic hypoparathyroidism but high in pseudo- hypoparathyroidism, which is also characterized by bony abnormalities. Idiopathic hypoparathyroidism Idiopathic hypoparathyroidism typically occurs in the young; the mean age at onset is 25 to 35 years. For this diagnosis to be made, there must be no FIGURE. Computed tomographic scan showing coarse evidence of renal insufficiency, rickets, osteoporosis, calcifications in the basal ganglia, thalami, and cerebellar or sprue.5,5 hemispheres bilaterally. Diffuse, linear calcifications are Neurologic manifestations. Idiopathic hypopa- present at the gray-white junction. rathyroidism is characterized by tetany and sei- zures. 3,5,7 Although psychiatric manifestations occur, they are not usually the presenting feature except in ate and started calcitriol (Rocaltrol) 0.25 jig by the elderly, in whom dementia appears to be a mouth four times a day. She was not experiencing prominent feature.8,9 In fact, all of the few reported any acute or life-threatening events secondary to cases in the elderly have been associated with psy- her hypocalcemia to require emergent calcium re- chiatric manifestations.4,7 placement. Her blood urea nitrogen (BUN) and Approximately 70% to 86% of all patients with creatinine levels returned to normal (BUN 16 idiopathic hypoparathyroidism experience seizures, mg/dL, creatinine 0.6 mg/dL) after rehydration; her compared with only 30% in all other forms of hy- calcium level increased to 7.8 mg/dL 48 hours after poparathyroidism.''' Tetany occurs in approxi- the addition of calcitriol. At discharge (9 days after mately 70% of all patients who have seizures due to admission and 4 days after the start of calcitriol) her hypoparathyroidism.' These seizures are frequently calcium level was 9.1 mg/dL. misdiagnosed as hysterical, as they may be precipi- During her hospital stay, the patient would sign tated by emotion and are difficult to control with inappropriately and had multiple spells of disorien- anticonvulsants.3 Trousseau's and Chvostek's signs tation.
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