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.     

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.     

Hypoparathyroidism and facial dysmorphism as main symptoms of 22q.11 deletion syndrome

A 12-year-old Japanese boy with mental retardation and facial dysmorphism developed frequent convulsions, and hypocalcemia due to hypoparathyroidism was recognized. Chromosomal analysis involving the fluorescence in situ hybridization method revealed a microdeletion of 22q11.2. However, other laboratory examinations revealed no cardiac anomaly, thymic hypoplasia, or cleft palate. It is well known that typical cases of 22q11 deletion syndrome have a cardiac anomaly, thymic hypoplasia and a cleft palate. However, the phenotype of 22q11 deletion syndrome is diverse, and hypoparathyroidism and facial dysmorphism have been reported in nine cases, including this case, associated with 22q11 deletion. This combination of clinical manifestations could be given another term, such as hypoparathyroidism-facial syndrome. Some hypoparathyroidism patients due to 22q11.2 deletion may be misdiagnosed as having idiopathic hypoparathyroidism, and a child diagnosed as having hypoparathyroidism should be examined for chromosomal 22q.11.2. deletion.     

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     

Hypoparathyroidism and pseudotumor cerebri: an infrequent clinical association

We report a patient with chronic, untreated idiopathic hypoparathyroidism who presented with papilledema and progressive deterioration of visual function. The papilledema resolved with treatment of the hypocalcemia. Visual acuity progressively improved as the serum calcium rose during treatment with vitamin D and calcium supplements. Lumbar puncture may also have contributed to the normalization of cerebrospinal fluid pressure and recovery of vision in this patient. The association of hypoparathyroidism and pseudotumor cerebri is rare, and a retrospective review of 41 patients with hypoparathyroidism admitted to two local general hospitals revealed no other cases.     

A case with temporal lobe epilepsy associated with hypocalcemia due to primary hypoparathyroidism]

We report a 30-year-old man with temporal lobe epilepsy associated with hypocalcemia due to primary hypoparathyroidism. He had frequent jamais vu since age 14 years, and later developed generalized convulsive seizures. It was initially controlled by phenytoin. The patient also had hypocalcemia due to primary hypoparathyroidism. The presence of jamais vu suggested the seizure focus in the mesial temporal area, being consistent with EEG finding, and it was most likely activated by the associated hypocalcemia. Control of hypoparathyroidism, and furthermore, selection of valproic acid as an antiepileptic drug which did not interfere with calcium metabolism were essential for this particular patient.     

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.     

Hypoparathyroidism: A rare treatable cause of epilepsy – report of two cases

Hypoparathyroidism occurs due to insufficient production of parathyroid hormone to maintain extracellular calcium levels within the normal range. The acute clinical symptoms and signs of hypoparathyroidism are those of hypocalcaemia, ranging from tingling and numbness of limb extremities to intractable seizures. Often seizures are mistaken for epilepsy. Though hypoparathyroidism is not uncommon, the diagnosis is often missed due to its unusual clinical manifestation. This is the first documented report with vitamin D, Parathormone levels and urinary biochemical parameters from India. We present two cases of hypoparathyroidism who presented with seizures along with a short review of literature.     

Reversible peripheral neuropathy in idiopathic hypoparathyroidism

We describe a 40-year-old male with idiopathic hypoparathyroidism presenting with tetany, proximal weakness, signs of hypocalcaemia including Chvostek and Trousseau's and diminished tendon reflexes in the upper and lower limbs. Electrophysiological studies revealed a sensory-motor neuropathy, predominantly axonal as evidenced by decreased CMAP amplitudes, with normal distal latencies-velocites, except for median nerve where a prolonged distal latency was observed. Serial nerve conduction studies were performed at repeated intervals for 2 years, while he received treatment for hypoparathyroidism (calcium and vitamin D supplementation). A progressive improvement in neuropathy both clinical and on electrophysiological studies was observed. Occurrence of peripheral neuropathy in hypocalcaemic states such as hypoparathyroidism and its reversibility after normalization of calcium homeostasis lend proof to the role of critical Ca2+ ion concentration in the normal functioning of the peripheral axons.     

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.     

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.     

Fahr's disease in postthyroidectomy hypoparathyroidism. A case report

The onset of Fahr's disease in a patient with postoperative hypoparathyroidism is described. The genesis of this rare pathological occurrence could be ascribed, in our opinion, to the combined action of "local" (precedent encephalic circulation disorders) and "general" factors (Ca and P metabolism disorders following postoperative hypoparathyroidism).     

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.     

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.     

Exacerbation of idiopathic paroxysmal kinesigenic dyskinesia in remission state caused by secondary hypoparathyroidism with hypocalcemia after thyroidectomy: Evidence for ion channelopathy

Most reported cases of paroxysmal kinesigenic dyskinesia (PKD) are idiopathic or familial; however, hypoparathyroidism is another unusual cause of secondary PKD. The pathomechanism of PKD remains poorly understood, and the association between idiopathic and secondary PKD remains an enigma, and has yet to be clearly elucidated. We recently encountered a patient with idiopathic PKD whose symptoms were aggravated by secondary hypoparathyroidism with hypocalcemia after having undergone a thyroidectomy. The patient's paroxysms were ameliorated by the normalization of serum calcium levels. The results discussed herein may provide support for the hypothesis that PKD is associated with neuronal ion regulation.     

An analysis of findings EEG and brain CT scans in patients with primary hypoparathyroidism]

10 cases of primary hypoparathyroidism showed abnormal findings on EEG. Among them 9 cases were also verified by CT scans. It was considered that the severity of abnormalities in primary hypoparathyroidism was not only influenced by the serum concentration of calcium, but also related with the severity and extent of calcification in the brain. These findings would be of significance with regard to the diagnosis, treatment and prognostic evaluation of primary hypoparathyroidism.     

Extensive brain calcification and progressive dysarthria and dysphagia associated with chronic hypoparathyroidism

An 81-year-old woman with a 13-year history of hypoparathyroidism developed dysarthria and dysphagia. Cranial computed tomography demonstrated extensive calcification involving the basal ganglia, corona radiata, and deep cerebellar structures. The cerebral small-vessel calcification that occurs in chronic hypoparathyroidism may produce the syndrome of progressive dysarthria and dysphagia.     

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.     

Secondary cervical dystonia in iatrogenic hypoparathyroidism associated with extensive brain calcifications

Cervical dystonia (CD) is usually idiopathic, without a known aetiology. Hypoparathyroidism, both primary and secondary, can be associated with brain calcifications and various clinical neurological features. Anecdotal evidence suggests that patients affected by hypoparathyroidism show a rapid-onset oral dyskinesia after use of neuroleptic drugs. We report the case of a 60-year-old woman with CD, iatrogenic hypoparathyroidism and extensive brain calcifications. On the basis of the clinical features and the localization of the brain calcifications we suppose that they may have played a role in the development of this CD. This case may prove to be, after a review of literature, the first report of CD secondary to iatrogenic hypoparathyroidism in a patient with extensive brain calcifications.     

Tonic-clonic seizures in a patient with primary hypoparathyroidism: a case report.

Hypoparathyroidism, a life threatening disorder, occurs when insufficient parathyroid hormone is produced to maintain extracellular calcium levels within the normal range. The acute clinical signs and symptoms of hypoparathyroidism are the same as those of hypocalcemia, ranging from tingling to intractable generalized tonic-clonic seizures; therefore, it can be mistaken for epilepsy. We report the case of a 36-year-old man who presented two tonic-clonic seizures, characterized by sudden loss of consciousness with a fall and diffuse tonic contractions and clonic jerks. At first diagnosis of epilepsy was established and therapy with valproate was commenced. In the following days, the patient presented typical signs of hypocalcemia and the diagnosis of hypoparathyroidism was made. In the 4 months follow up, antiepileptic drug therapy was reduced until suspension and calcium supplementation was initiated. We briefly review the most recent reports in the literature.