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.     

Autoimmune polyendocrine syndrome type I and brain calcinosis

Autoimmune polyendocrine syndrome (APS) is a rare disorder. One of the possible associated endocrinopathies in APS is hypoparathyroidism. We describe brain calcifications secondary to hypoparathyroidism in family members with APS and compare clinical manifestations, the extent of brain calcifications on CT scans and the result of PET–FDG scans. We found extensive brain calcifications and striatal hypometabolism in PET–FDG scan in the only symptomatic member of the family, which supports the assumption that extensive brain calcification and the presence of hypometabolism in PET–FDG scan are likely to be found in symptomatic patients with brain calcifications.     

Basal ganglia calcifications in postoperative hypoparathyroidism: a case with unusual characteristics

A patient is described with post-thyroidectomy hypoparathyroidism and basal ganglia calcifications. The patient presented with a tonic-clonic seizure. The calcifications shown on CT scan were extensive in the basal ganglia, the bifrontal periventricular white matter and even in the brainstem. The white matter with calcifications displayed a high signal intensity on MRI, probably due to a different stage of the calcifying process in the white matter than in the basal ganglia. A severe dystonic reaction was observed after phenothiazine therapy and is discussed.     

Psychopathological alterations in cases of symmetrical basal ganglia sclerosis

Psychopathological alterations caused by symmetrical basal ganglia sclerosis of different etiologies are described, involving cases with parathyroid gland/hormone dysfunction (some of them familial), patients after thyroidectomy, and patients with basal ganglia calcification of uncertain etiology. Initial symptomatology in a group of 62 patients is reported; chronic symptoms in another group of 35 patients were evaluated. Estimates of volume of the basal ganglia calcifications were made, in addition to precise topographical localizations by CT. In 40% the initial symptoms noted were psychiatric, compared with 50% who first presented neurological symptoms. In the group of chronic cases practically all showed intellectual impairment. There was a marked preponderance of organic affective syndromes (initially 21%, chronic 65%): the affective chronic patients can be subdivided into 37% depressive, 20% bipolar, 11% manic cases. We could find no direct relationships with regard to etiology, localization, volume or symptoms, except that extensive calcifications occur after parathyroid hormone deficiencies due to thyroidectomy and lead to more severe mental deterioration.     

Calcification of the basal ganglia: computerized tomography and clinical correlation

During a 1-year period, 4219 consecutive computerized tomograms (CT) were reviewed for basal ganglia calcification; 14 patients with such calcification were identified. Calcifications on CT scan were bilateral in 12 of these cases and unilateral in 2. All bilateral calcifications were symmetric. The globus pallidus was the site of calcification in 13 of the 14 patients. Bilateral dentate nucleus calcification was seen in one patient. Skull radiograms were normal in all but one. Patients had diverse symptoms that were often explained by other findings, suggesting that calcifications may be coincidental and that basal ganglia calcification may not be a nosologic entity. Disturbances of calcium metabolism were not found in these patients, minimizing the pathophysiologic significance of altered calcium metabolism and the need for extensive endocrinologic evaluation. The finding of basal ganglia calcification alone does not justify invasive diagnostic procedures. Extrapyramidal signs may be associated with basal ganglia calcification; parkinsonism associated with basal ganglia calcification differs from idiopathic parkinsonism in being resistant to levodopa therapy.     

Utilization of neuropsychiatric diagnostic tests for general hospital patients with mental disorders.

OBJECTIVE: The author's goal was to determine the frequency and distribution of neuropsychiatric diagnostic tests provided to general hospital patients with mental disorders. METHOD: Data from the 1989 National Hospital Discharge Survey were analyzed to determine the number, proportion, and general characteristics of 11,628 discharged patients with primary diagnoses of mental disorders who underwent computerized tomography (CT) scanning of the head, EEG, and magnetic resonance imaging (MRI) of the brain. RESULTS: Of the discharged patients with mental disorders, 5.1% had received CT scans, 2.8% had received EEGs, and 0.7% had received MRI. These rates were below the rates for patients discharged with primary diagnoses of neurological disorders but above the rates for patients discharged with primary diagnoses of other medical disorders. Among the patients discharged with mental disorder diagnoses, the likelihood of receiving a CT scan or an EEG was greater if the primary diagnosis was an organic disorder or if the secondary diagnosis was a medical disorder. Patients over age 65 were also more likely to have received a CT scan. Hospital size and location had a modest influence on the likelihood of receiving a CT scan or EEG, but the ownership of the hospital and the patient's source of payment were not significant influences. CONCLUSIONS: Neuropsychiatric diagnostic testing is selectively utilized in the routine treatment of general hospital psychiatric inpatients. Clinical variables rather than institutional or financial variables appear to be the most powerful predictors of which patients are selected to receive these tests.     

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.     

Basal ganglia calcification on computer tomographic scan

Computerised tomography of the brain was used to demonstrate basal ganglia calcification which may be of an insufficient degree to be seen on skull X-ray.
Cases referred for CT scan from hospitals in Singapore for various reasons over a period of 12 months were studied for basal ganglia calcification. There was a startlingly high incidence of 1.5%, as 47 cases (all except 2 were Chinese) showed such calcification. In 42 cases there was no evidence of basal ganglia calcification on skull X-ray. 16 cases showed neurological affection, fits being the commonest manifestation.
An unexpected finding was that no case had abnormality of calcium metabolism or evidence of hypoparathyroidism or pseudohypopara-thyroidism.
The CT scan is very sensitive in demonstrating minimal basal ganglia calcification and our impression is that such calcification is common.     

早发型和晚发型情感性障碍的遗传效应比较

为了解早发型和晚发型情感性障碍间是否存在遗传效应的差异，对同时符合中国精神疾病分类方案与诊断标准第２版和美国精神障碍诊断和统计手册第４版情感性障碍诊断标准的１８０例患者，以３０岁为界，划分为早发组（１１３例）和晚发组（６７例）。所得资料行单因素分析，用多基因阈值理论进行遗传率的估算。结果显示，早发组有精神疾病家族史多，一级亲属中情感性障碍的发病风险率高，一级亲属的加权平均遗传率高，脑部ＣＴ显示有器质性改变者少，与晚发组比较，差异均有显著性。提示二者存在着遗传异质性。     

CT scan findings in mild head trauma: a series of 2,000 patients

The present study describes the cranial computed tomography (CT) scan findings of 2,000 cases of mild head trauma (HT) in Curitiba, Southern Brazil. The mean age of the entire series was 30.8 +/-19 years. The overall male to female ratio was 2:1. The most common causes of head injury were interpersonal aggression (17.9%), falls (17.4%), automobile accidents (16.2%), falls to the ground (13.1%) and pedestrian injuries (13 %). Alcohol intoxication was associated with HT in 158 cases (7.9%). A normal CT scan was seen in 60.75% (1215) and an abnormal CT scan in 39.25% (785) of patients. Out of 785 abnormal CT scan, 518(65.9%) lesions were related to HT. The most common CT scan HT related findings were: soft tissue swelling (8.9 %), skull fractures (4.3 %), intracranial and subgaleal hematomas (3.4% and 2.4 %), brain swelling (2 %) and brain contusion (1.2%). Out of 785 abnormal CT scans, 267 (34.1%) lesions were not related to head trauma. Incidental CT scan findings included brain atrophy (5.9%), one calcification (5.2%) several calcifications (2.4%) (probably neurocysticercosis in most cases), ischemic infarct (1.9%) and leukoaraiosis (1.3%). These findings showed the importance of CT scan examination in mild head injuries. Further studies to identify mild HT patients at higher risk of significant brain injury are warranted in order to optimize its use.     

Computerized tomography in the diagnostic evaluation of multiple sclerosis.

Sixty-six patients with a tentative or certain diagnosis of multiple sclerosis (MS) were examined with cranial computerized tomography (CT). Abnormalities found in 19 (29%) included discrete areas of decreased white matter density, enlarged ventricles, cortical atrophy, and focal areas of contrast enhancement. The presence of white matter lucencies and ventricular dilatation correlated with an increased incidence of diffuse hyperreflexia and mental impairment. Since half the patients with contrast-enhancing lesions were clinically stable, their lesions may represent acute asymptomatic plaques. Abnormalities on CT scan were observed more often in older patients whose disease had lasted longer and was clinically more definite. Since findings were sparse in early, less definite disease, the use of CT as a purely diagnostic tool is limited.     

Basal ganglia calcifications in children]

We report the study of four children with bilateral basal ganglia calcifications (BGC) visualized on CT scan. Epilepsy was the clinical manifestation of three patients whose laboratory investigation revealed abnormal calcium metabolism. The first aim of this paper is to call attention to a treatable entity that can cause epileptic syndromes in infancy and childhood. The second purpose is to review the literature comparing with our fourth child who presented encephalopathy with BGC.     

伴钙化与囊变的脑白质病二例临床、影像学表现及相关病理学分析

目的 探讨伴钙化与囊变的脑白质病(LCC)的临床、影像学表现以及相关病理学特点,以进一步增强对该病的认识和诊断能力.方法 2例患者均行CT和(或)MRI平扫,例2另加MRI增强扫描.2例均行实验室血清生化及钙、磷和甲状旁腺素等检查,例2行腰椎穿刺及脑脊液检查;均经手术切除相关囊性病灶并行组织病理学检查.结果 CT平扫2例均见脑内广泛钙化并伴囊性灶(例1位于右额叶、右尾状核旁;例2位于左顶叶、左丘脑).MRI增强扫描囊性灶边缘示轻度环状强化.组织病理学示囊壁小血管变形呈血管瘤样改变,伴玻璃样变性、钙化及含铁血黄素沉积;周围脑组织血管见纤维素样坏死伴腔内血栓形成,呈小动脉炎改变;脑组织部分变性伴胶质增生、Rosenthal纤维形成及出血.实验室生化检查各指标均正常.例2腰穿示颅内压增高(350 mm H_2O,1 mm H_2O=0.0098 kPa).结论 LCC的首发年龄及症状各异,其影像学上的钙化形态及分布不对称.脱髓鞘及血管源性水肿可能共同参与了脑白质异常信号的改变.     

Capgras delusion in the elderly: The evidence for a possible organic origin

We report the case of a 77-year-old right-handed woman with early dementia of the Alzheimer type who developed the paranoid delusion that her husband had been replaced by an imposter (Capgras phenomenon). Her CT scan showed mild generalized cortical atrophy of the parieto-occipital cortex. We reviewed the cases of 45 patients over 60 years who showed a Capgras delusion. Twenty-five suffered from dementia or other chronic organic mental disorders, 20 were paranoid, 14 were at least moderatley depressed and 12 disorientated. CT scans (reported in 17 cases) showed global cerebral atrophy in 12 patients. Right hemisphere infarcts have been described in two elderly patients with Capgras delusions. Focal brain atrophy in Alzheimer type dementia has hitherto not been reported in association with this delusional symptom. We conclude from these observations that marked cerebral lesions and specific neuropsychological deficits are not required for the development of the Capgras phenomenon, which can occur in various functional or organic psychosis of old age.     

Long-term outcome in aqueductal stenosis

In this study, 78 patients with aqueductal stenosis were submitted to detailed neurodevelopmental assessment with a follow-up of 5–25 years. Sixty-eight percent of patients were categorized as normal; they either attended normal school courses or had regular jobs. Among these, 34% had some motor abnormalities (ataxia, mild hemiparesis, visual disturbances). Twenty-four percent (19 cases) were moderately disabled (trainable retardation) and 8% (6 cases) were severely handicapped. Epilepsy was observed in 13% of the cases. Incidence of recurrent and generalized seizures paralleled neurodevelopmental outcome (5% in normal, 16% in moderately disabled and 50% in severely disabled patients). Endocrine dysfunctions were evident in 28% of the cases and were characterized by precocious or delayed puberty, amenorrhea and somatic underdevelopment. No patient with ventricular enlargement and a cortical mantle width below 20 mm showed a good outcome. Large ventricles were compatible with normal mental development when compensated with a corresponding cranial vault enlargement. In patients with normal mental status and motor abnormalities, longterm CT scan findings revealed the presence of focal brain abnormalities (poroencephaly, brain atrophy, calcifications, extracerebral collections).Presented at the Consensus Conference: Hydrocephalus '92, Assisi, Italy, 26–30 Aprill 1992     

Cranioplasty for isolated trigonocephaly with developmental disorder]

We reported 50 cases of mild to moderate trigonocephaly (most isolated type) treated by cranioplasty. All of them had clinical symptoms such as severe hyperactivity, speech delay, inability to communicate with others, self-mutilation (head banging), irritability, temper tantrum and mental retardation. Pre-operative CT scan and MRI showed no abnormal findings in the brain except for constricted frontal lobes. The 3 D-CT scan showed the most important diagnostic findings: a ridge of the metopic suture and narrow anterior fossa. TcECD SPECT was performed on 43 patients, and demonstrated in 31 cases some degree of decreased cerebral blood flow (CBF), mainly in the bilateral frontal lobes. Post-operatively, most patients improved to some degrees. The results were compared to those of trigonocephaly patients without cranioplasty. The operated group showed better improvement in the above clinical symptoms, especially, hyperactivity, indifference to others, understanding of verbal communication, self-mutilation, irritability and temper tantrum. The post-operative SPECT represented the increased CBF in 30 out of the 31 cases. MRI and CT scan revealed expanded frontal lobes. Thus, cranioplasty may alleviate the symptoms of patients with mild to moderate trigonocephaly and developmental disorders.     

A case of idiopathic hypoparathyroidism with extrapyramidal signs and insulin-dependent diabetes mellitus

A case of a 29-year-old woman with idiopathic hypoparathyroidism was reported. There were neither endocrine nor neurological disorders among her family, except for her mother's hearing loss. She had been suffering from insulin-dependent diabetes mellitus since 21 years of age, and was noticed to be hard of hearing for several years, but never been examined. At the age of 27, choreic movement on her left upper limb and gait disturbance appeared. A year before admission, gait disturbance gradually developed and she could not walk any more. On admission, her height was 137.2 cm and her weight 36.5 kg. She had a round face, uneven teeth and borderline metacarpal sign on her right hand. On neurological examination, Parkinsonism, bucco-lingo-masticatory dyskinesia and bilateral extensor planter reflex were present, but tetany was not observed anywhere. Serum calcium was 3.9 mEq/l, and serum phosphorus 5.3 mEq/l. A CT scan of brain revealed calcifications in the bilateral basal ganglia and thalami, low density area in the left putamen, and atrophy of both caudate nuclei. Serum PTH was less than 100 pg/ml. Ellsworth-Howard's test showed hyperresponsiveness in the secretion of urinary phosphorus and cyclic-AMP. Other endocrinological studies showed no abnormality except for hyporesponsiveness in the secretion of insulin on glucose tolerance test. On the basis of these results, a diagnosis of idiopathic hypoparathyroidism with insulin-dependent diabetes mellitus was made. Administration of alfacalcidol returned serum calcium and phosphorus to normal with considerable clinical benefit. Parkinsonism was gradually improved and she became to be able to walk with a cane after one year of treatment. But buco-lingo-masticatory dyskinesia were not reduced.(ABSTRACT TRUNCATED AT 250 WORDS)     

Neurocysticercosis in childhood. I. Clinical and laboratory diagnosis

Clinical and laboratory findings among 34 children (age range from 15 months to 13 years) with neurocysticercosis were reviewed. The main symptoms were: intracranial hypertension, 21 cases (62%); epilepsy, 20 cases (59%); hemiplegia, 4 cases (12%). Computed tomography (CT) in 33 children showed typical brain active cysts in 26 patients (79%) and calcifications in 2 (6%). The complement fixation reaction or the indirect immunofluorescent test for cysticercus antibody were positive in CSF in 77% (20 out of 26 patients) and serum in 78% (18 out of 23 patients). CSF pleocytosis was found in 57% of the cases (15/26 patients) with eosinophil cells in 27% (7/26 patients). The most efficient diagnostic test was CT scan of the head, and was confirmed immunologically by measurement of cysticercus antibody titers in CSF and serum.     

Magnetic resonance imaging in agenesis of the corpus callosum

The recent use of computed tomography (CT) scan is providing an easy diagnoses of agenesis of corpus callosum which had been difficult to diagnose only by clinical signs and symptoms. Since, more neuropsychological studies on agenesis of corpus callosum are being done, clinical details of agenesis of corpus callosum are being clarified. We examined magnetic resonance imaging (MRI) on 3 patients who were suspected to have agenesis of corpus callosum by CT scan. And we studied the usefulness of MRI in agenesis of corpus callosum. Case 1. We studied a thirty-two-year-old right-handed man with a one-year history of amyotrophic lateral sclerosis. He had no callosal disconnection syndrome. CT scan on horizontal view showed dilated third ventricle, the dilatation of the occipital horns and wide separation of the lateral ventricles. On coronal view, CT scan showed the absence of the corpus callosum, the concave mesial borders of the lateral ventricle, the dilatation of the third ventricle and its abnormal dorsal extent(bat-wing appearance). On sagittal MRI (the inversion recovery technique), the corpus callosum was absent and the convolutions and sulci were arranged radically centering the third ventricle (radial distribution). But the anterior commissure was preserved. Case 2. We studied a fourty-one-year-old right-handed man with a seven-year history of hyper ventilation syndrome. He had no callosal disconnection syndrome. CT scan showed the similar findings as those of case 1. On sagittal MRI (the inversion recovery technique), the corpus callosum was absent, and the convolutions and sulci showed radial distribution. But the anterior commissure was preserved.(ABSTRACT TRUNCATED AT 250 WORDS)     

Dural metastasis from prostatic adenocarcinoma mimicking chronic subdural hematoma

Dural metastasis is rare. In most reported cases, brain CT scan findings are mistaken for subdural hematoma or meningioma. We present here a 72-year-old male with a history of headache and progressive mental status changes. Brain CT scans suggested chronic subdural hematoma. However, the only surgical findings were diffuse thickening of the dura and sclerosis of the temporal bone. Histopathology revealed metastatic prostatic carcinoma. As the surgical approach and prognosis of chronic subdural hematoma and metastatic tumors are completely different, the differential diagnosis of these diseases is very important. A contrast-enhanced brain CT scan is recommended for patients who could possibly have dural metastases.