Post Graduate Institute of Medical Training and Research, Clinic of Endocrinology, Chandigarh, India
Post Graduate Institute of Medical Training and Research, Clinic of General Surgery, Chandigarh, India
J Clin Res Pediatr Endocrinol. 2020 Nov 25;12(4):432-438. doi: 10.4274/jcrpe.galenos.2020.2019.0181. Epub 2020 Mar 4.
Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity characterized by subcortical vasogenic edema presenting with acute neurological symptoms. Common precipitating causes include renal failure, pre-eclampsia/eclampsia, post-organ transplant, and cytotoxic drugs. Hypercalcemia is a rare cause of PRES; most cases occur in the setting of severe hypercalcemia secondary to malignancy or iatrogenic vitamin D/calcium overdose. Primary hyperparathyroidism (PHPT), as a cause of PRES, is an oddity. We report two cases of adolescent PHPT presenting with generalized tonic-clonic seizures and altered sensorium. On evaluation, both had hypertension, severe hypercalcemia (serum calcium 14.1 mg/dL and 14.5 mg/dL, respectively) and elevated parathyroid hormone levels. Magnetic resonance imaging (MRI) revealed T2/fluid-attenuated inversion recovery hyperintensities located predominantly in the parieto-occipital regions, suggestive of PRES. Identification and excision of parathyroid adenoma led to the restoration of normocalcemia. Neurological symptoms and MRI changes improved subsequently. An extensive literature search revealed only four cases of PHPTassociated PRES; none of them being in the pediatric/adolescent age group. The predominant clinical manifestations were seizures and altered sensorium. All had severe hypercalcemia; three had hypertension at presentation, while one was normotensive. Parathyroid adenomectomy led to normalization of serum calcium and resolution of neurological symptoms and radiological changes. Thus, severe hypercalcemia, although rare in PHPT, can lead to hypercalcemic crisis precipitating acute hypertension that can result in cerebral endothelial dysfunction with the breakdown of the blood-brain barrier, culminating in PRES. We therefore recommend that serum calcium levels should be checked in all patients with PRES and that PHPT be regarded as a differential diagnosis in those with underlying hypercalcemia.
后部可逆性脑病综合征（PRES）是一种临床放射学实体，其特征为皮质下血管源性水肿，表现为急性神经症状。常见的诱发原因包括肾衰竭、先兆子痫/子痫、器官移植后和细胞毒性药物。高钙血症是 PRES 的罕见原因；大多数病例发生在恶性肿瘤或医源性维生素 D/钙过量导致的严重高钙血症的情况下。甲状旁腺功能亢进症（PHPT）作为 PRES 的病因较为罕见。我们报告了两例青少年 PHPT 表现为全身性强直阵挛性发作和意识改变。评估时，两人均有高血压、严重高钙血症（血清钙分别为 14.1mg/dL 和 14.5mg/dL）和甲状旁腺激素水平升高。磁共振成像（MRI）显示 T2/液体衰减反转恢复高信号主要位于顶枕叶区域，提示 PRES。甲状旁腺腺瘤的识别和切除导致了正常钙水平的恢复。随后，神经症状和 MRI 改变得到改善。广泛的文献检索仅发现 4 例与 PHPT 相关的 PRES 病例；他们都不是在儿科/青少年年龄段。主要的临床表现是癫痫发作和意识改变。所有患者均有严重高钙血症；3 例患者在就诊时存在高血压，而 1 例患者血压正常。甲状旁腺腺瘤切除术导致血清钙正常，神经症状和影像学改变缓解。因此，尽管在 PHPT 中罕见，但严重高钙血症可导致高钙血症危象，引发急性高血压，导致脑内皮功能障碍，破坏血脑屏障，最终导致 PRES。因此，我们建议在所有 PRES 患者中检查血清钙水平，并将 PHPT 视为潜在高钙血症患者的鉴别诊断。
