Unusual Syndrome Research Articles

An Unusual Paraneoplastic Syndrome of Synchronous Bladder Tumor and Prostate Cancer: Polymyositis

Introduction: Polymyositis (PM) is a type of inflammatory myopathy that is associated with a broad range of malignant disorders. An association of PM with synchronous carcinoma of the bladder and prostate is extremely rare. Case: A 65-year-old man admitted to hematology with complaints of severe progressive weakness of lower extremities, hematuria and irritative urinary symptoms lasting for a month. The hemogram and erythrocyte sedimentation rate were normal. ALT was normal but AST was 405.56 U/l. There was marked elevation of serum creatine kinase (CK) and lactate dehydrogenase, which were 14,065.15 U/l and 1267.50 U/l, respectively. PSA was 4.28 and DRE was positive. The abdominal ultrasound revealed a 24 × 20 mm soft tissue echogenicity lesion at the right wall and a 35 × 21 mm soft tissue echogenicity lesion at the left wall of the bladder. The rest of the abdominal viscera were normal. Computed tomography found, two 6 mm solid lesions at left anterolateral and a 18 × 12 mm solid lesion at inferoanterior bladder wall in addition to the above findings. Patient counseled to us. We resected all of the bladder masses with transurethral (TUR-BT) way and pathology revealed T2 high grade bladder tumor. Two days after TUR-BT, we performed a trans rectal ultrasonography guided prostate biopsy and pathology revealed a Gleason 3 + 4 prostate cancer. EMG showed sensorimotor polyneuropathy at the lower extremities, sustaining polymyozitis. Biopsy of the right peroneus brevis muscle showed no vasculitis with low grade neurologic changes. We offered to perform a radical cystoprostatectomy operation but the patient prefered chemotherapy. Two months after his initial presentation at the second cure of the chemotherapy all muscle weaknesses showed a dramatic regression. Conclusion: This case report indicates that both bladder carcinoma and prostate cancer should be kept in mind in elderly PM patients presenting with lower urinary tract symptoms and hematuria.

Encephalopathy with status epilepticus during sleep: Unusual EEG patterns

PurposeTo retrospectively analyze the electroclinical characteristics, etiology, treatment, and prognosis of patients with epileptic encephalopathy with status epilepticus during sleep (ESES) with unusual EEG features and to corroborate if this series of patients is part of the ESES syndrome. MethodCharts of 17 patients with typical clinical manifestations of the ESES syndrome with focal ESES of non-REM sleep at onset and during the focal ESES phase, or bilateral synchronic and asynchronic ESES with a symmetric or asymmetric morphology, continuous or subcontinuous and sometimes multifocal paroxysms with or without slow-wave activity during slow sleep seen between 2000 and 2012 were analyzed. ResultsMean patient follow-up from onset was 7.5 years. An idiopathic cause was found in seven patients, a structural cause in eight, and etiology was unknown in the remaining two. The median age at onset of the unusual ESES syndrome was 7 years. During the ESES phase, 15 children developed new seizure types, negative myoclonus was observed in seven patients, positive myoclonus in five, and absences in nine. Six patients had motor impairment, two had auditory verbal agnosia, and two had motor speech impairment. Attention deficit hyperactivity disorder was observed in four, aggressiveness in six, memory deficit in two, and impaired temporospatial orientation in four.The patients with focal ESES in the frontal region showed behavioral disturbances and/or motor deterioration, and in those with temporo-occipital involvement the dominant clinical manifestations were language and/or behavioral disturbances. ConclusionOur patients with typical clinical manifestations of ESES syndrome but with unusual EEG patterns may be variants of this syndrome.

The extracellular domain of myelin oligodendrocyte glycoprotein elicits atypical experimental autoimmune encephalomyelitis in rat and Macaque species.

Atypical models of experimental autoimmune encephalomyelitis (EAE) are advantageous in that the heterogeneity of clinical signs appears more reflective of those in multiple sclerosis (MS). Conversely, models of classical EAE feature stereotypic progression of an ascending flaccid paralysis that is not a characteristic of MS. The study of atypical EAE however has been limited due to the relative lack of suitable models that feature reliable disease incidence and severity, excepting mice deficient in gamma-interferon signaling pathways. In this study, atypical EAE was induced in Lewis rats, and a related approach was effective for induction of an unusual neurologic syndrome in a cynomolgus macaque. Lewis rats were immunized with the rat immunoglobulin variable (IgV)-related extracellular domain of myelin oligodendrocyte glycoprotein (IgV-MOG) in complete Freund’s adjuvant (CFA) followed by one or more injections of rat IgV-MOG in incomplete Freund’s adjuvant (IFA). The resulting disease was marked by torticollis, unilateral rigid paralysis, forelimb weakness, and high titers of anti-MOG antibody against conformational epitopes of MOG, as well as other signs of atypical EAE. A similar strategy elicited a distinct atypical form of EAE in a cynomolgus macaque. By day 36 in the monkey, titers of IgG against conformational epitopes of extracellular MOG were evident, and on day 201, the macaque had an abrupt onset of an unusual form of EAE that included a pronounced arousal-dependent, transient myotonia. The disease persisted for 6–7 weeks and was marked by a gradual, consistent improvement and an eventual full recovery without recurrence. These data indicate that one or more boosters of IgV-MOG in IFA represent a key variable for induction of atypical or unusual forms of EAE in rat and Macaca species. These studies also reveal a close correlation between humoral immunity against conformational epitopes of MOG, extended confluent demyelinating plaques in spinal cord and brainstem, and atypical disease induction.

Discrepant serum and urine β-hCG results due to production of β-hCG by a cribriform-morular variant of thyroid papillary carcinoma

BackgroundAlthough patients with medullary thyroid cancer are known to present with paraneoplastic hormone production, this is much less common with papillary thyroid cancer. MethodsWe present a patient with the cribriform morular variant of papillary thyroid cancer in association with familial adenomatous polyposis who developed a positive pregnancy test in the absence of known pregnancy. The patient had developed vaginal bleeding, and her laboratory testing was characterized by elevated serum human chorionic gonadotropin (β-hCG) concentrations, but negative qualitative urine results. After a thorough gynecological evaluation to exclude unexpected normal, ectopic, or molar pregnancy, we pursued an evaluation for other sources of β-hCG production. ResultsWe showed that the elevated serum β-hCG concentrations were not the result of heterophile antibody interferences, and ultimately we proved that her recurrent tumor produced the ectopic β-hCG. This is the first report of β-hCG production by papillary thyroid cancer. Thus, the possibility of ectopic production of β-hCG by papillary thyroid cancer needs to be included in the differential diagnosis of elevated hCG concentration in the absence of pregnancy. ConclusionsThis study of an unusual paraneoplastic syndrome highlights the importance of investigating discrepancies in the clinical laboratory.

Mutations in GRHL2 Result in an Autosomal-Recessive Ectodermal Dysplasia Syndrome

Grainyhead-like 2, encoded by GRHL2, is a member of a highly conserved family of transcription factors that play essential roles during epithelial development. Haploinsufficiency for GRHL2 has been implicated in autosomal-dominant deafness, but mutations have not yet been associated with any skin pathology. We investigated two unrelated Kuwaiti families in which a total of six individuals have had lifelong ectodermal defects. The clinical features comprised nail dystrophy or nail loss, marginal palmoplantar keratoderma, hypodontia, enamel hypoplasia, oral hyperpigmentation, and dysphagia. In addition, three individuals had sensorineural deafness, and three had bronchial asthma. Taken together, the features were consistent with an unusual autosomal-recessive ectodermal dysplasia syndrome. Because of consanguinity in both families, we used whole-exome sequencing to search for novel homozygous DNA variants and found GRHL2 mutations common to both families: affected subjects in one family were homozygous for c.1192T>C (p.Tyr398His) in exon 9, and subjects in the other family were homozygous for c.1445T>A (p.Ile482Lys) in exon 11. Immortalized keratinocytes (p.Ile482Lys) showed altered cell morphology, impaired tight junctions, adhesion defects, and cytoplasmic translocation of GRHL2. Whole-skin transcriptomic analysis (p.Ile482Lys) disclosed changes in genes implicated in networks of cell-cell and cell-matrix adhesion. Our clinical findings of an autosomal-recessive ectodermal dysplasia syndrome provide insight into the role of GRHL2 in skin development, homeostasis, and human disease.

A case series of late postoperative capsular block syndrome treated by Nd:YAG laser posterior capsulotomy

AbstractPurpose To evaluate the clinical characteristics of the unusual late postoperative capsular block syndrome (CBS) and the effect of neodymium:yttrium‐aluminium‐garnet (Nd:YAG) laser posterior capsulotomy on visual acuity, refractive error as well as its possible complicationsMethods Retrospective cohort study. Twelve eyes of 11 patients with late CBS who had undergone Nd:YAG laser posterior capsulotomy were reviewed. A complete examination including Scheimpflug camera and anterior segment optical coherence tomography (AS‐OCT) imaging had been performed before and after posterior capsulotomyResults BCVA increased in 11 cases (91.7%). Only one eye showed a 0.5 diopter hyperopic shift following posterior capsulotomy. There were no post‐laser complications such as increased IOP or cystoid macular edema. In all patients, the posterior capsule was vaulted posteriorly and could not be seen clearly. AS‐OCT confirmed CBS in the studied cases, demonstrating a distended capsular bag. Rotating Scheimpflug imaging examination showed the white substance located behind the IOLConclusion Late CBS is usually not accompanied by shallow anterior chamber, forward IOL displacement or raised IOP. Nd:YAG laser posterior capsulotomy is a useful treatment and the prognosis of patients with this complication appears favorable. Although the AS‐OCT measurements are easier to get than Scheimplug imaging, both are useful to visualize the distended capsular bag containing the white material and the IOL position

Femoral hypoplasia- Unusual Facies Syndrome or Femoral Facial Syndrome with Radioulnar Synostosis

DOI: http://dx.doi.org/10.3329/bjch.v38i1.20028 Bangladesh J Child Health 2014; VOL 38 (1) : 48-50

Importance of Early Cranioplasty in Reversing the "Syndrome of the Trephine/Motor Trephine Syndrome/Sinking Skin Flap Syndrome".

The "Motor Trephine Syndrome (MTS)" also known as the "Sunken brain and Scalp Flap Syndrome" or the "Sinking Skin Flap Syndrome (SSFS)" or the "Syndrome of the trephined" is an unusual syndrome in which neurological deterioration occurs following removal of a large skull bone flap. This syndrome is associated with sensorimotor deficit and neurological deterioration following decompressive craniectomy which is performed for various neurosurgical conditions involving cerebral swelling causing mass effect. The neurological deterioration can be exacerbated or precipitated by CSF diversion procedures like a Ventriculo-Peritoneal shunt. It was the objective of this study to observe if any improvement in the patient's condition, or if any beneficial effects in his sensorimotor deficit could be gained by performing an early cranioplasty as against after the usual delay of one to two years normally allowed for post-craniectomy cases. A 52 year old male suffered severe head injury in a road traffic accident and underwent a craniectomy and contusectomy of the left Fronto-Temporo-Parietal (FTP) region for treatment of Acute Subdural hematoma (SDH) as well as hemorrhagic and non-hemorrhagic contusions of the brain with severe mass effect. On recovery from this acute event he was bed bound, on tracheostomy, his GCS was E4VTrM4 with residual right sided hemiparesis. Three months later, he developed Hydrocephalus for which a Right Ventriculo-Peritoneal (V-P) shunt was performed. Following this procedure, severe depression of the skin/scalp flap occurred and the neurological recovery was not as expected. He was diagnosed as a case of "Syndrome of the trephined". An immediate Cranioplasty was performed, on the third month following the craniectomy procedure, in an attempt to resolve the rapidly deteriorating neurological status of the patient. In the case presented, following the early Cranioplasty which was performed within three months of the initial craniectomy, the patient's neurological condition and cognitive functions showed a remarkable, immediate and dramatic improvement. The early Cranioplastic repair led to a remarkable clinical recovery of the patient, with improvement in the cognitive behavior and motor deficit with a rapid reversal of the sensorimotor paresis, reflecting an improvement in brain perfusion. Patients with the classical "Motor trephine syndrome/ Sinking skin flap syndrome" following large craniectomy defects, may hugely benefit from an early cranioplasty procedure, with a reversal of features of this syndrome and early recovery of their neurological and cognitive functions. Hence, an early cranioplasty can serve as a therapeutic procedure, rather than merely a cosmetic one.

Compound heterozygous CORO1A mutations in siblings with a mucocutaneous-immunodeficiency syndrome of epidermodysplasia verruciformis-HPV, molluscum contagiosum and granulomatous tuberculoid leprosy.

Coronin-1A deficiency is a recently recognized autosomal recessive primary immunodeficiency caused by mutations in CORO1A (OMIM 605000) that results in T-cell lymphopenia and is classified as T(-)B(+)NK(+)severe combined immunodeficiency (SCID). Only two other CORO1A-kindred are known to date, thus the defining characteristics are not well delineated. We identified a unique CORO1A-kindred. We captured a 10-year analysis of the immune-clinical phenotypes in two affected siblings from disease debut of age 7 years. Target-specific genetic studies were pursued but unrevealing. Telomere lengths were also assessed. Whole exome sequencing (WES) uncovered the molecular diagnosis and Western blot validated findings. We found the compound heterozygous CORO1A variants: c.248_249delCT (p.P83RfsX10) and a novel mutation c.1077delC (p.Q360RfsX44) (NM_007074.3) in two affected non-consanguineous siblings that manifested as absent CD4CD45RA(+) (naïve) T and memory B cells, low NK cells and abnormally increased double-negative (DN) ϒδ T-cells. Distinguishing characteristics were late clinical debut with an unusual mucocutaneous syndrome of epidermodysplasia verruciformis-human papilloma virus (EV-HPV), molluscum contagiosum and oral-cutaneous herpetic ulcers; the older female sibling also had a disfiguring granulomatous tuberculoid leprosy. Both had bilateral bronchiectasis and the female died of EBV+ lymphomas at age 16 years. The younger surviving male, without malignancy, had reproducibly very short telomere lengths, not before appreciated in CORO1A mutations. We reveal the third CORO1A-mutated kindred, with the immune phenotype of abnormal naïve CD4 and DN T-cells and newfound characteristics of a late/hypomorphic-like SCID of an EV-HPV mucocutaneous syndrome with also B and NK defects and shortened telomeres. Our findings contribute to the elucidation of the CORO1A-SCID-CID spectrum.

Late postoperative capsular block syndrome: a case series studied before and after Nd:YAG laser posterior capsulotomy.

To evaluate the clinical characteristics of the unusual late postoperative capsular block syndrome (CBS) and the effect of Nd:YAG laser posterior capsulotomy on visual acuity and refractive error as well as its possible complications. In this retrospective cohort study, 12 eyes of 11 patients with late CBS who had undergone Nd:YAG laser posterior capsulotomy were reviewed. A complete ophthalmic examination including Scheimpflug camera and anterior segment optical coherence tomography (AS-OCT) imaging had been performed before and after posterior capsulotomy. The mean time between cataract surgery and posterior capsulotomy was 4.7 ± 1.5 years (range 3-9 years). Best-corrected visual acuity increased in 11 cases (91.7%). Only one eye showed a 0.5-D hyperopic shift following posterior capsulotomy. There were no postlaser complications such as increased intraocular pressure (IOP), severe inflammation, cystoid macular edema, or retinal detachment. In all patients, the posterior capsule was vaulted posteriorly and could not be seen clearly. The AS-OCT confirmed CBS in the studied cases, demonstrating a distended capsular bag. Rotating Scheimpflug imaging examination showed the white substance located behind the intraocular lens (IOL). Late CBS is usually not accompanied by shallow anterior chamber, forward IOL displacement, or raised IOP. Nd:YAG laser posterior capsulotomy is a useful treatment and the prognosis of patients with this complication appears favorable. Furthermore, although AS-OCT measurements are easier to obtain than rotating Scheimpflug imaging examination, both are useful to visualize the distended capsular bag containing the white material and the IOL position.

A rare case of diastematomyelia with hemivertebrae and intradural teratoma

Diastematomyelia, or split cord malformation, a complete or incomplete sagittal division of the neural axis into halves, is seen in association with many other congenital anomalies. Among these anomalies, intradural spinal teratoma is extremely rare. Diastematomyelia is a well-recognized although unusual clinical syndrome in children, but it is rarely reported in the adult. We report a case of 19 year old girl who presented with pain and distal left-leg weakness as well as neurogenic claudication for 1 month. The patient underwent radiological examinations, and diastematomyelia and an intradural lumbar teratoma were diagnosed. she underwent surgery with definite anaesthesia management. This is the first case of an adult who simultaneously presented with diastematomyelia and an intradural teratoma with hemivertebrae. We hereby discuss the anaesthetic plan and management of the same. I. Case report: A 19-year-old lady weighing 40 kg and measuring 133 cm in height presented with uterine prolapse for Fothergills surgery with anteroposterior repair. There was no history suggestive of any major illness in the past. She had undergone tubal ligation under general anaesthesia uneventfully four months prior to this surgery. She had hair growth on her lumbar vertebrae region. There was no history suggestive of a neurological deficit, breathlessness or limitation of lower limbs or spine. One month back had a h/o fall from a parapet, following which she developed lower limb weakness, inability to walk, motor activity was reduced.

What's in the Literature?

The spectrum of congenital myasthenic syndromes continues to expand, and recent studies found mutations in enzymes that glycosylate peptides. Affected patients have early onset of a limb-girdle pattern of weakness, and the disorder is treatment responsive. Another article addressed quinine use for slow channel syndrome. There is an additional report on the use of rituximab in acquired myasthenia gravis, and the risk of developing cancer with azathioprine therapy for myasthenia gravis is also covered. Regarding amyotrophic lateral sclerosis, we review articles addressing the utility and safety profile of botulinum injections for sialorrhea and possible associations with autoimmune diseases. Moving to peripheral nerve, we address reports on small fiber neuropathy as a cause of muscle cramps, reexamine clinical and electrodiagnostic features of childhood Guillain–Barré syndrome, and review a recent article on an unusual syndrome of postirradiation lower motor neuron syndrome affecting the cauda equina. Finally, recent reports show that mutations in the skeletal muscle ryanodine receptor may be a common cause of rhabdomyolysis and exertional myalgias and another cause of axial myopathy. Advances in clinical and basic science concepts of myotonic dystrophy are also reviewed.

Acute idiopathic blind spot enlargement syndrome associated with choroidal neovascularization

Clinical caseA 17-year-old female presented with photopsia and a sudden loss of visual field in left eye (OS), with previous contralateral choroidal neovascularization. The examination suggested an acute idiopathic blind spot syndrome. The progress without treatment was favorable, with a reduction in the scotoma and without a worsening of her visual acuity. DiscussionThis case report is about an unusual and benign syndrome, typical of young women. Differential diagnosis must be made between the evanescent white dot syndrome and the acute zonal occult outer retinopathy. To our knowledge, this is the first published case associated with choroidal neovascularization, a fact that leads us to question its benignancy.

The Syndrome of Hypoparathyroidism, Deafness, and Renal Anomalies

ObjectiveWe review the syndrome of hypoparathyroidism, deafness, and renal anomalies (HDR syndrome). MethodsThe current understanding and relevant literature pertaining to the background, genetic considerations, clinical features, prognosis, and treatment of HDR syndrome are reviewed. ResultsThe combination of hypoparathyroidism, deafness, and renal anomalies constitutes an unusual syndrome associated most commonly with haploinsufficiency in GATA3, which encodes a transcription factor that binds to the (A/T) GATA (A/G) consensus DNA sequence. Sensorineural hearing loss is the most consistently expressed clinical feature, being present in almost all affected individuals, and the combination of hypoparathyroidism and hearing impairment occurs in well over 90% of those affected, with various renal anomalies being the most heterogeneous feature of the classic triad. We characterize, in tabular form, the individual cases described in the literature and propose a classification scheme based on the presence or absence of renal anomalies. We also include the specific genetic abnormality and renal anomaly associated with each individual case. ConclusionHDR syndrome is a heterogeneous syndrome most commonly associated with GATA3 haploinsufficiency. (Endocr Pract. 2013;19:1035-1042)

Self-injurious behaviour in children: A treatable catatonic syndrome

There is an excellent reason for pub-lishing a paper on an unusual paediat-ric syndrome – self-injurious behaviour (SIB) – in a general psychia-try journal: it is among the few paedi-atric conditions that are highly treatable. The repetitive stereotypic catatonic movements in children with autism and intellectual disabilities, which can have such injurious conse-quences, respond readily to such standard treatments of catatonia as benzodiazepines and electroconvul-sive therapy (ECT). The authors of this paper have discovered this in their respective domains of inquiry: Lee Wachtel, leading a neurobehav-ioural service in a paediatric hospital, and Edward Shorter, who ascertained that in many historical accounts of paediatric SIB other symptoms of cat-atonia were present as well, reinforc-ing Wachtel’s hypothesis of SIB as a form of catatonia. This hypothesis thus has two important pillars of sup-port: the readiness with which SIB responds to anti-catatonic remedies in the clinic today, and the historical evidence that SIB fits in with larger syndromes of catatonia.Thanks to the efforts of Max Fink and the circle of scholars building upon his findings, catatonia has expanded steadily from a subtype of schizophrenia into an independent syndrome that may occur in a variety of psychiatric and non-psychiatric ill-nesses. The reinsertion of the catato-nia diagnosis into psychiatry is being officially recognized in the newly-released DSM-5. We feel that this recognition represents one of the most important developments in psy-chiatric nosology today.SIB is classically defined as any self-inflicted act resulting in bodily harm. It afflicts approximately 5–30% of indi-viduals with intellectual disability (ID), and has negative effects on physical health as well as sharply impairing global psychosocial functioning.It is remarkable to discover that the inclusion of self-injury in the cata-tonic spectrum is not new. In fact, strong evidence of this concomitance can be traced in the international psy-chiatric literature beginning in the late 19th century. The historical evidence of catatonia in self-injury patients offers fascinating parallels to modern self-injurious presentations, raising the central question as to how this rich historical knowledge escaped incorporation into classic models of SIB assessment and treatment, partic-ularly when modern medicine has possessed a safe and efficacious treat-ment for catatonia, namely ECT, for nearly eight decades.

Síndrome de aumento agudo idiopático de mancha ciega asociado a neovascularización coroidea

Clinical caseA 17 year old female consulting due to photopsia and a sudden loss of visual field in left eye (OS), with previous contralateral choroidal neovascularization. The examination suggested an acute idiopathic blind spot syndrome. The progress without treatment was favorable, with a reduction in the scotoma and without a worsening of her visual acuity. DiscussionThis case report is about an unusual and benign syndrome, typical of young women. Differential diagnosis must be made between the evanescent white dot syndrome and the acute zonal occult outer retinopathy. To our knowledge, this is the first published case associated with choroidal neovascularization, a fact that leads us to question its benignancy.

Exacerbated Type II Interferon Response Drives Hypervirulence and Toxic Shock by an Emergent Epidemic Strain of Streptococcus suis

Streptococcus suis, a major porcine pathogen, can be transmitted to humans and cause severe symptoms. A large human outbreak associated with an unusual streptococcal toxic shock-like syndrome (STSLS) was described in China. Albeit an early burst of proinflammatory cytokines following Chinese S. suis infection was suggested to be responsible for STSLS case severity, the mechanisms involved are still poorly understood. Using a mouse model, the host response to S. suis infection with a North American intermediately pathogenic strain, a European highly pathogenic strain, and the Chinese epidemic strain was investigated by a whole-genome microarray approach. Proinflammatory genes were expressed at higher levels in mice infected with the Chinese strain than those infected with the European strain. The Chinese strain induced a fast and strong gamma interferon (IFN-γ) response by natural killer (NK) cells. In fact, IFN-γ-knockout mice infected with the Chinese strain showed significantly better survival than wild-type mice. Conversely, infection with the less virulent North American strain resulted in an IFN-β-subjugated, low inflammatory response that might be beneficial for the host to clear the infection. Overall, our data suggest that a highly virulent epidemic strain has evolved to massively activate IFN-γ production, mainly by NK cells, leading to a rapid and lethal STSLS.

An Unusual Mucocutaneous Syndrome with Sensorineural Deafness Due to Connexin 26 Mutations

Mutations of the GJB2 gene, which encodes connexin 26, are related to a range of conditions associated with sensorineural deafness and keratinization disorders. We present the case of a newborn girl with sensorineural deafness, erythematous hyperkeratotic plaques on intertriginous areas, and parakeratosis on the oral and esophageal mucosa. She had an F142L mutation in exon 1 of the GJB2 gene, which was described previously in a patient with a similar phenotype.

Hypercalcemia in a child with juvenile granulosa cell tumor of ovary: Report of an unusual paraneoplastic syndrome and review of the literature

► Hypercalcemia is an extremely rare paraneoplastic syndrome in children. ► Small cell carcinoma is the commonest ovarian tumor associated with hypercalcemia. ► Small cell carcinoma must be ruled out because of poor prognosis. ► We report the only third case of JGCT associated with paraneoplastic hypercalcemia.

A case of unusual acute coronary syndrome

Pheochromocytoma is a rare tumor that usually develops ahead of the neuroectodermal chromaffin cells of the adrenal medulla, but it may arise anywhere within plexus of sympathetic adrenergic nerves. Headache, palpitations, tremor, excessive sweating, abdominal pain, and hypertensive paroxysm are the common clinical presentations of the tumor, but it has also been reported several cardiac symptoms.