Hypocalcemic generalised seizures as a manifestation of iatrogenic hypoparathyroidism months to years after thyroid surgery.

Hypoparathyroidism is a relatively common side effect of a thyroidectomy and leads to hypocalcemia. Carpopedal spasm and tetany are typical manifestations and usually occur within weeks after surgery. The first signs can be less typical and include movement disorders such as chorea, as well as symptoms of increased intracranial pressure or epileptic seizures. We describe two cases with generalised tonic-clonic seizures as the first manifestation of postoperative hypoparathyroidism, appearing months and years after thyroidectomy. Iatrogenic hypoparathyroidism needs to be considered in the differential diagnosis of adult-onset, generalised, tonic-clonic seizures even if the thyroidectomy was performed years earlier.     

Idiopathic hypoparathyroidism with extensive brain calcification and persistent neurologic dysfunction

Idiopathic hypoparathyroidism is an uncommon cause of movement disorders. The following case illustrates the persistence of a parkinsonian gait 2 years after the restoration of normal serum calcium levels. The extensive calcifications in the brain presumably account for this as well as for the persistent mild dementia. The importance of identifying hypoparathyroidism early in the course is graphically illustrated.     

Depression as a manifestation of latent chronic hypoparathyroidism.

The exact cause of depression in cases of hypoparathyroidism is not known. We report the first case of an elderly patient with a long history of major depression as a complication of an undiagnosed chronic hypoparathyroidism following surgery on a parathyroid adenoma. Her depression was completely eliminated by calcium supplementation therapy to restore the calcium homeostasis in serum. As it is well known that disturbances in the endocrine hypothalamus-pituitary-thyroid system might be consistent findings of depressive disorders concerning neuroendocrinological alterations, this case report and review of literature strongly supports our claim that also parathyroid diseases like chronic hypoparathyroidism, even in its latent form, might be a relevant factor in the development of depressive symptoms.     

Neonatal hypocalcemic seizures in siblings exposed to topiramate in utero

We describe two siblings with neonatal hypocalcemic seizures whose mother took topiramate during both pregnancies. Apart from hypocalcemia, the patients had no identifiable etiology for their seizures. Although biochemical data suggested that the hypocalcemia was caused by hypoparathyroidism, no disorders typically associated with this condition were identified in the patients. We propose that topiramate exposure in utero led to hypoparathyroidism and subsequent hypocalcemia via effects on protein kinase A signaling, resulting in hypocalcemic seizures. Neonates exposed to topiramate in utero should be monitored for hypocalcemic seizures.     

Calcinose striatopallidodentelée,hypoparathyroïdie et atteinte neurologique : étude de série

IntroductionThe respective roles of hypocalcemia and intracerebral calcifications in the occurrence of various neurological manifestations in hypoparathyroidism is not entirely clear. Nevertheless, therapeutic and prognostic implications are important.ObjectivesWe analyze the neurological clinical aspects observed in hypoparathyroidism and correlate them to the biological calcium abnormality and radiological CT scan findings. We also compare these results with data reported in the idiopathic form of striatopallidodentate calcinosis.PatientsThe neurological clinical, CT scan findings and outcome have been retrospectively studied in patients recruited during 13 years (2000–2012) for neurological features associated with hypoparathyroidism or pseudohypoparathyroidism.ResultsTwelve patients with primary hypoparathyroidism (n = 5), secondary to thyroidectomy (n = 4) and pseudohypoparathyroidism (n = 3) were studied. The sex-ratio was 1 and mean age was 39 years. All patients had a tetany, 60% had epilepsy, associated in one patient with “benign” intracranial hypertension; 50% had behavioral changes. Response to calcium therapy was excellent for all these events. Moderate cognitive deficit was noted in three patients (25%), parkinsonism in two patients and hyperkinetic movement disorders in one other. These events were not responsive to calcium therapy and were more common in cases of extensive brain calcifications and in patients who had pseudohypoparathroidism.CommentsThis study suggests that, in patients with hypoparathyroidism, epilepsy and psychiatric disorders are induced by hypocalcemia and reversible after its correction. Cognitive and extrapyramidal impairment seem to be related to the progressive extension of intracerebral calcification, particularly in patients with a late diagnosis. In patients with pseudohypoparathyroidism, this finding is different because of the contribution of other factors, specific to this disease.     

An unusual case of paroxysmal kinesigenic dyskinesia.

Paroxysmal kinesigenic dyskinesia (PKD) is an uncommon neurological disorder characterised by abnormal episodic brief movements induced by sudden movements of the body. The recognition and understanding of this disorder has increased over the past few decades. While most cases are idiopathic, the association of PKD with various disorders, including metabolic abnormalities has also been reported. We report an interesting case of a 52 year old male who presented with PKD manifesting as subtle facio-brachial movements and apraxia of eyelid opening (ALO) secondary toidiopathic hypoparathyroidism.     

Contribution of CT scan to the diagnosis of Fahr''s syndrome

Of 7040 patients who were examined with CT scan for various reasons, 72 (1.02%) showed symmetrical intracranial calcifications. On the basis of the extent of the calcifications, the patients were divided into 3 groups of which the first (10 cases), with extensive calcifications, showed clear findings of hypoparathyroidism; the second (4 cases), with less extensive calcifications, showed borderline laboratory findings and the third (58 cases), with calcifications only in the internal part of the globus pallidus, had no relation to any disturbance of the parathyroid function. The limited percentage (4.2%) of calcifications detected by ordinary cranial X-rays was discussed as compared to those diagnosed by CT scan; this is due to the weakening of the relationship which existed in the past between hypoparathyroidism and calcifications. Patients with extensive calcifications do not seem to have a significantly higher proportion of neurological impairment (35.8%) than patients who showed limited calcifications (34.5%). On the other hand, patients with extensive calcifications showed more frequent mental disorders (50%) compared to those who showed limited calcifications (34.5%). The proportion of mental disorders is still higher in patients with dilatation of the subarachnoid spaces (56.7%) compared to those who do not show dilatation (23.8%). Finally, of the 72 patients with calcifications, 15 (20.8%) showed extrapyramidal syndrome, possibly due to calcium salt deposits in the metasynaptic dopamine receptors while 13 (18%) were taking anticonvulsant drugs which, apart from influencing the metabolism of calcium, also influence the activity of alkaline phosphatase and are involved in the creation of calcifications.     

Neurocysticercosis associated with hypoparathyroidism and Fahr's disease (?): report of a case

The author reports a case of neurocysticercosis and hypoparathyroidism in a 28 year-old female with bilateral symmetric basal ganglia calcification demonstrable by C.A.T.-Scan. A brief review of the literature is made in order to show why the eponym "Fahr's disease" should not be applied to this case.     

Normalization of personality characteristics in patients with incapacitating anxiety disorders after capsulotomy

The personality characteristics of 24 consecutive patients undergoing psycho-surgery for incapacitating anxiety disorders were assessed prospectively using a self-report personality inventory. The main findings were: absence of negative personality changes after surgery, significant postoperative changes towards normalization on the majority of the scales, and significant symptomatic relief in 80% of the cases. The changes on scales reflecting anxiety proneness were conspicuous in patients suffering from "pure" anxiety disorders, as compared with those suffering from obsessive-compulsive disorder (OCD). In OCD patients, correlations were obtained between changes in brain metabolism studied with positron emission tomography and changes in personality scores. It is concluded that negative personality changes are not likely to occur after capsulotomy.     

Short report: Paroxysmal choreoathetosis as a presenting symptom in idiopathic hypoparathyroidism

A patient with idiopathic hypoparathyroidism presenting with spells of paroxysmal choreoathetosis is described. The possible mechanisms by which hypoparathyroidism induces choreoathetosis and other extrapyramidal motor dyfunctions are discussed. The need for screening patients with extrapyramidal disease for hypoparathyroidism is stressed.     

甲状旁腺功能减低的神经精神表现

目的 对甲状旁腺功能减低（甲旁低）患者以神经精神症状为首发的临床特点加认报道。方法 对原因不明，常规治疗无效的所谓“癫痫”和其它精神临床表现患者进行有关甲旁低的全面检查。结果 发现５例甲旁低患者中的特发性和继发性甲旁低分别为３例和２例，经早期，合理治疗后，其神经精神临床表现恢复，结论 对原因不明，常规治疗无效的所谓“癫痫”和其它神经精神临床表现患者应做有关甲旁低的全面检查，以早期诊疗，争取全面康复     

Nonorganic and Organic Psychiatric Disorders in Patients after Epilepsy Surgery

This study aimed at describing preoperative psychiatric morbidity in a consecutive series of 70 epilepsy patients who were surgically treated and to analyze postoperative psychiatric morbidity and predisposing factors. Nonorganic (DSM-III-R) and organic (Lindqvist-Malmgren diagnostic system) psychiatric morbidity was prospectively assessed preoperatively and during the first two postoperative years. At presurgical evaluation 44.3% of the patients had a psychiatric diagnosis (nonorganic in 14.3%, organic in 38.6%). The most common nonorganic diagnosis was major depression; the most common organic diagnosis was Astheno-Emotional disorder (AE disorder). During the first two postoperative years 68.1% of the patients received some psychiatric diagnosis. The most common nonorganic diagnoses were anxiety and depressive disorders (AD disorders) in 36.2%; the most common organic diagnosis was AE disorder in 52.2%. Patients with a preoperative history of AD disorders or AE disorder had a significantly higher risk of postoperative AD disorders (P < 0.01 and P < 0.001 respectively). Laterality, type of resection, histopathological diagnosis, or outcome were not significantly related to postoperative psychiatric morbidity. The importance of psychiatric assessment, including organic psychiatric disorders, is emphasized.     

Spectrum of neurological manifestations of idiopathic hypoparathyroidism and pseudohypoparathyroidism

We describe clinical, biochemical, radiological profile, and treatment outcome in 97 patients with idiopathic hypoparathyroidism seen over a period of 18 years. Of the 97 patients, 78 (80%) had idiopathic hypoparathyroidism and 19 (20%) had pseudohypoparathyroidism. The mean age±standard deviation (SD) at presentation was 28.7±14.1 years. There were 52 males, the mean lag time from first reported symptom to diagnosis was 5.9±5.2 years and the mean (±SD) follow-up was 1.8±0.4 years. The most common presenting manifestation was carpopedal spasm in 68 (70%) patients, followed by paresthesia and seizures in 52 (54%) patients. The mean (±SD) serum calcium and inorganic phosphate concentrations were 6.1±1.5 mg/dl and 6.3±1.5 mg/dl, respectively. The most common imaging abnormality noted was basal ganglia calcification followed by cerebral cortex and cerebellum calcification. More than one-third of patients were on various antiepileptic drugs including phenytoin. In addition to oral calcium and active vitamin D (calcitriol), twenty-six patients (27%) also required hydrochlorothiazide. The important finding in our study was long lag time from the first reported symptom to diagnosis. Phenytoin was the drug in almost one- third of our patients with seizures. Practicing clinicians should have high index of suspicion of diagnosis hypoparathyroidism in the appropriate clinical states to avoid the morbidity associated with hypoparathyroidism. Phenytoin should be avoided in patients with hypoparathyroidism and seizures.     

Hypocalcemia, hypoparathyroidism, and organic anxiety syndrome

The author describes a case of functional hypoparathyroidism with hypocalcemia that developed in a patient after ablation of a parathyroid adenoma. The patient's clinical presentation was characterized by a severe anxiety state. The literature on the neuropsychiatric manifestations of hypoparathyroidism indicates that anxiety states are not uncommon in this condition and suggests that hypoparathyroidism should be considered in the differential diagnosis of organic anxiety syndrome.     

Parkinsonism,basal ganglia calcification and epilepsy as late complications of postoperative hypoparathyroidism

Summary A patient with post-thyroidectomy hypoparathyroidism, basal ganglia calcification, parkinsonism and seizures is reported. The parkinsonism was resistant to levodopa therapy but was not significantly improved by the correction of hypoparathyroidism. Previously reported cases are discussed, as well as the relationship between hypoparathyroidism, calcification of basal ganglia, parkinsonism and epilepsy.     

A case of adult-onset spike-wave stupor associated with hypoparathyroidism and hyperostosis frontalis interna (HFI)

A 34-year-old women without history of seizures was admitted to our hospital because of the diminished responsiveness and the repeated blinking for two days. Her past history showed neither surgery nor inflammation of the thyroid gland. General physical examination was normal. On neurological examination, the patient was blinking frequently and staring without any responsiveness. However, she occasionally became able to respond correctly to verbal orders, such as "open your eyes", "open your mouth", "stand up" and so on, which occurred abruptly just like the switch-on. She was otherwise normal neurologically. The laboratory data showed hypocalcemia, hyperphosphatemia, decreased level of parathyroid hormone and normal renal function, indicating the presence of idiopathic hypoparathyroidism. EEG showed the continuous generalized 2-4 Hz spike and wave complexes with the maximum intensity on frontal lobes. Skull roentgenograms and MRI CT of the head disclosed hyperostosis frontalis interna (HFI) and the compression of superior medial frontal lobes by the HIF. SPECT also showed the diminished circulation and hypometabolism in the superior frontal lobes. Based on clinical and EEG findings, the diagnosis of spike-wave stupor was made. She was successfully treated with valproic acid and ethosuximide. Spike and wave complexes on EEG completely disappeared after administration of alfacalcidole. It is assumed that both hypoparathyroidism and HFI were deeply involved in the development of spike-wave stupor in this adult case.     

Crises épileptiques révélant des anomalies du métabolisme phosphocalcique

Hypocalcemia due to hypoparathyroidism produces a broad spectrum of clinical manifestations, but overt symptoms may be sparse. One unusual presentation is onset or aggravation of epilepsy in adolescence revealing hypoparathyroidism. This situation can lead to delayed diagnosis, with inefficacity of the antiepileptic drugs. We report five cases of adolescence-onset epilepsy with unsuccessful antiepileptic therapy, even with gradually increasing dose. Physical examination revealed signs of hypocalcemia, confirmed biologically. Full testing disclosed the origin of the seizures: hypoparathyroidism in three patients and pseudohypoparathyroidism in the other two. In four of five patients, computed tomography showed calcification of the basal ganglia, defining Fahr's syndrome. The patients were treated with oral calcium and active vitamin D (1-alphahydroxy vitamin D3). Seizure frequency progressively decreased and serum calcium levels returned to normal. These cases illustrate the importance of the physical examination and of routine serum calcium assay in patients with new-onset epileptic seizures in order to detect hypocalcemia secondary to hypoparathyroidism.     

Role of the parathyroid glands in the pathogenesis of intracerebral calcifications

Investigations of calcium-phosphate metabolism were carried out in a group of 11 children and adults with intracerebral calcifications. It was possible to isolate three different pathogenetic types of calcifications in the striatum and dentate nuclei in the cerebellum. The authors suggest restriction of the term "Fahr's syndrome" to cases without true of false hypoparathyroidism. The assessment of the calcium-phosphate metabolism, and particularly, the test with parathyroid hormone, seem to be an indispensable element in the differential diagnosis of this type of intracerebral calcifications.     

Psychiatric disorders following and preceding temporal lobectomy]

We analysed pre- as well as post-operative psychiatric disorders in thirty eight patients with temporal lobe epilepsy. While postoperative paranoid disorders were closely correlated with preoperative acute interictal psychoses, episodes of postictal psychoses prior to surgery were associated with postoperative mood disorders. A good prognosis of postoperative mood disorders was stressed. The literature search supported the predominance of right-sided lobectomy in patients with de novo psychoses after surgery. The relationship between left-sided lobectomy and postoperative mood disorders needs further amplification and remains tentative. We stressed the need for a prophylactic psychotherapy to surgical candidates to cope with unrealistic wishes to get relieved from all the difficulties in their lives after temporal lobectomy.     

Two cases of epileptiform attacks due to hypocalcemia in a child with hypoparathyroidism

Two cases are described of epileptiform convulsive seizures and consciousness disturbances in girls. The diagnosis of hypocalcaemia due to hypoparathyroidism was considerably delayed, and was made only when irreversible brain damage developed. The pathological mechanism, principles of diagnosis and treatment of hypoparathyroidism are discussed.