We report a 46-year-old man with idiopathic hypoparathyroidism who presented with gradually prograding cognitive impairment and generalised tonic clonic epilepsy. Computed tomography (CT) and magnetic resonance imaging (MRI) of brain showed calcification in the basal ganglia, thalamus and cerebral white matter. Calcium level was low and phosphor was increased in serum, but parathormon level was low. Following introduction of replacement therapy, cognitive functions improved as well. After treatment with calcium and vitamin D supplementation he showed clinical, cognitive impairment and laboratorial improvements.
Hypoparathyroidism can result from lack or decreased secretion of parathormon (PTH) or from defective responsiveness of effector organs to PTH. Neurological signs of hypoparathyroidism include symptomatic or latent tetany, seizures, hemiparesis, difficulty in speaking, ischemic attacks, in case of basal ganglia and cerebellar calcifications also choreoatetosis, parkinsonism and cerebellar syndrome (1-3).
Clinical, laboratory and radiological features of cases of hypoparathyroidism make basis of diagnostics of the primary disease with various neurological signs. Laboratory findings revealed severe hypocalcemia and hyperphosphatemia, and serum low levels of parathyroid hormone. Computed tomography (CT) and magnetic resonance imaging (MRI) of brain showed calcifications in the basal ganglia, thalamus, cerebral white matter and cerebellum (4-7).
We report a very rare case of a patient with hypoparatiroidism who manifested cognitive impairement and epilepsy seizure. We compare our case with others in medical literature.
46-year man is hospitalised because of two generalised tonic clonic seizures. The patient's friends who escorted him reported gradual decrease of his intallectual functions over the last two years. he consumes alcohol moderately and smieks up to 60 cigarettes a day. Neurological signs are dysmetria and tongue bite. The Chvostek's and the Trousseau's signs are negative. The basic blood laboratory tests are normal. The heart and lungs x-ray shows lime saturation of medial and apical, right handside, parts of the pleura.
EEG showed the continuous generalized 2-4 Hz spike and wave complexes. CT of the brain shows infra- and supratentorially, temporo-parieto-occipitally, large lacunar and punctiform intracerebral calcifications. The basal ganglia and the corona radiata are completely saturated with lime salts. The ventricular structures are centrally located and normally shaped, Figure 1.
Figure 1. CT of the brain – difusely situated numerpus calcificates (periventricular, subkortical, centrum semiovale)
MRI of the brain shows bilateral, in the area of the coronae radiatae, globus pallidus, and posterior parts of the talamusa, lamilar calcificates that indicate lime saturation of the basal ganglia blood vessel walls. More marked are cortical atrophic changes and periventricular leukomalation, Figure 2.
Figure 2. MRI of the brain – T2 whighting – periventricular and subcortical calcifications
The upper abdomen sonography is normal. Electromiographical test for tetany is negative. Echographic examnation shows the thyroid gland to be normally homogenous, of isoechognous structure, free of nodes. The parathyroid glands are echographycally normal. Biomicroscopy established bilateral presenile cataract. Psychological tests indicate marked mental deterioration, dominating in the fields of thinking, planning and organisation of mental activities, with damaged flexibility. Simple visual perception is preserved, marked deficites are noticed in complex spatial functions, visoperceptive organisation and visomotor coordination disorders, with mental and motoric deceleration. There are markedly decreased learning processes with recalling difficulties, but memory is preserved at the recognition level. There is high anxiety and impulsivity, and lowered frustration tolerance.
After treatment with calcium and vitamin D supplementation he showed clinical, neurophysiological and laboratorial improvements.
Clinical, laboratory and radiological features of our case of hypoparathyroidism presenting with pure neurological disease are described.
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 extremities to intractable seizures (7). Seizures may occur at any age and are usually generalized tonic-clonic ones (1, 8). Generalized tonic-clonic seizures are often secondary signs of brain lesion, but rarely makes a cause of hypoparathyroidism. Our patient had two sequential seizures, which indicates a brain leisure, requiring radiological examination, CT and MRI. CT better defined the site and extent of the calcifications which may be found in both idiopathic and postoperative hypoparathyroidism, in symptomatic or asymptomatic patients. MRI seems to be capable of depicting the various stages of calcium deposition on the basis of the presence of reduced or increased SE T2 weighted images (3). CT and MRI of the brain of our patient agrees with the above and adds to the clinical diagnosis. Laboratory findings revealed severe hypocalcemia and hyperphosphatemia, and parathyroid hormones levels were low in serum. This is in line with other authors' researches (4, 5). The usual EEG findings in patients with genaralised tonic clonic seizures, as well as the caratact, have already been described in patients with brain calcificates caused by hypoparathyroidism. Clinical, laboratory and radiological features of our case of hypoparathyroidism are further completed with neuropsychiatric tests that showed a cognitive impairment. Pathogenesis of cognitive and motor changes in Fahr's disease and hyperparathyroidism with calcification of the brain are based in a dysfunction of corticobasal connections and their interhemispheric relations. This defines a subcortical dementia secondary to mineral deposits in subcortical structures (10, 11). The cognitive impairment and neurological deficit incidence in patients with intracerebral calcification caused by hypoparathyroidism is very much present, according to other authors' reseasrches (12, 13). Joint cognitive impairment and epilepsy seizure in adult patients require additional radiological and laboratory examinations for a possible hypoparthyroidism.
Paprocka J, Jamroz E, Wackermann-Ramos A, Sokol M, Marszal E.
Neurological picture and 1H MRS in 4 children with hypoparathyroidism.
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Avrahami E, Cohn DF, Feibel M, Tadmor R. MRI demonstration and CT
correlation of the brain in patients with idiopathic intracerebral calcification.