Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare primary headache syndrome first described in 1978. We report on a 43-year-old man with a 10 year history of SUNCT in whom a pituitary macroadenoma was eventually detected. His pain rapidly improved with medical treatment of ...

BACKGROUND: The short-lasting unilateral neuralgiform headache associated with conjunctival injection and tearing or SUNCT syndrome was first described in the 1970s. This paper is the first in the literature that describes the successful use of stereotactic radiosurgery (SRS) using a non-invasive frameless technique, targeting both the trigeminal nerve and the ...

Red ear syndrome (RES) is characterised by attacks of unilateral or bilateral burning ear pain associated with erythema. Primary and secondary forms have been described. Primary RES appears to have a frequent association with primary headaches especially migraine. Here, we describe the case of a woman with short-lasting unilateral neuralgiform ...

We report a 16-year-old adolescent with 2 episodes of focal neurological deficits, pseudomigrainous headache, and lymphocytic pleocytosis due to the syndrome of transient headache and neurological deficits with cerebrospinal fluid (CSF) lymphocytosis (HaNDL), also known as pseudomigraine with CSF pleocytosis. Review of the literature identifies 13 additional cases of HaNDL ...

Sturge-Weber syndrome is known to be associated with migraine attacks and prolong aura even without cerebral infarction. We report the case of a 36-year-old woman with type III Sturge-Weber syndrome developing with prolonged left homonymous hemianopsia after an intractable migraine-like headache and becoming a permanent visual field defect at 18-month ...

The most critical element in headache evaluation is the history. The targeted history differentiates primary from secondary headaches and provides a realistic list of conditions associated with secondary headache. Several of these conditions present with specific physical findings, such as papilledema, Horner's syndrome, or a cranial nerve palsy. The targeted ...

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is classified under trigeminal autonomic cephalalgias. This rare headache syndrome is infrequently associated with secondary pathologies. In this paper the authors report on a patient with paroxysmal left retroorbital pain with associated autonomic symptoms of ipsilateral conjunctival injection ...

SMART syndrome (stroke-like migraine attacks after radiation therapy) is a rare condition that involves complex migraines with focal neurologic findings in patients following cranial irradiation for central nervous system malignancies. Little is known about the mechanisms behind the disorder, making successful treatment challenging. We report 2 new cases of SMART ...

Complementary and alternative medicine (CAM) is widely used by both physicians and patients with primary headache syndromes. Despite a considerable number of articles addressing CAM in primary headache syndromes, the overall evidence for CAM is still poor. The aim of this review was to give an overview of the current ...

Mucopolysaccharidosis (MPS) Type VI, or Maroteaux-Lamy syndrome, is characterized by a deficiency of the enzyme arylsulfatase B (ASB). In patients with this disorder, craniocervical compression, carpal tunnel syndrome, and communicating hydrocephalus are common. Traditionally, hydrocephalus occurring in patients with MPS VI has been treated with shunt placements. Considering obstruction of ...

Chronic headache is known to be aggravated by fasting and fasting even triggers headache among those not suffering from chronic headache. Headache is also aggravated during Ramadan in which Muslim people do not eat, drink, or smoke from dawn to sunset for about one month in a year. Headaches mainly ...

OBJECTIVE/BACKGROUND.: Some headache syndromes have few cases reported in the literature. Their clinical characteristics, pathogenesis, and treatment may have not been completely defined. They may not actually be uncommon but rather under-recognized and/or underreported. METHODS.: A literature review of unusual headache syndromes, searching PubMed and ISI Web of Knowledge, was ...

This case report describes a 13-year-old girl whose family requested a referral from the pediatrician for Child and Adolescent Mental Health Services in order to understand her recent onset of bizarre behavior. On assessment, she was found to have episodes of complex audiovisual hallucinations and panic attacks with intervals of ...

A 24-year-old man with a history of Klippel-Trenaunay-Weber syndrome presented with severe headache and neck pain. Work-up revealed subarachnoid hemorrhage and evidence of multiple intracranial aneurysms. The patient was treated with open surgical clipping of his ruptured aneurysm and is currently doing well.

Headache and seizures are common presentations to the acute medical unit. We report a case of posterior reversible encephalopathy syndrome (PRES) in a 73 yr old woman with a history of uncontrolled hypertension who had recently discontinued one of her antihypertensive drugs; such cases are rare but pose a diagnostic ...

Turner syndrome (TS) (approximately 1:5,000 births) and craniopharyngioma (CP) (1:50,000 children) are both rare conditions. We present three cases of TS with CP, an association not previously described. Visual failure, poor growth or headache led to MRI diagnosis of CP. Whilst two had evidence of hypopituitarism at diagnosis of CP, ...

An 11-year-old girl had four episodes of fever in a year, lasting 7-10 days and associated with headache and neck stiffness. She had a long history of recurrent urticaria, usually preceding the fevers. There was also a history of vague pains in her knees and in the small joints of ...

Inclusion body myositis (IBM) belongs to the group of idiopathic inflammatory myopathies. It is a poorly understood disease, which affects skeletal muscles. IBM usually occurs as an isolated condition, but in some cases, it may be associated with another autoimmune disorder, Sjögren's syndrome. We report a case of a 47-year-old ...

Background:Red ear syndrome is characterised by episodic unilateral erythema of the ear associated with pain and burning sensation.Patients and methods:We describe the case of a young woman with daily, frequent, recurrent episodes of red ear syndrome in the absence of any structural lesions, primary headache disorder or obvious triggers. We ...

The syndrome of transient headache and neurologic deficits associated with cerebrospinal fluid lymphocytosis (HaNDL) is characterized by 1 or more episodes of severe headache, transient neurologic deficits, and lymphocytic pleocytosis in the cerebrospinal fluid. It is a benign and self limited disorder seldom reported in pediatric age. We report the ...

Manganese is a trace element and a cofactor of many enzymes, so it is essential for physiologic functioning, but it is also a neurotoxin at high doses. Manganism is most often caused by occupational exposure. It is manifested by a myriad of signs and symptoms ranging from the neurasthenia syndrome, ...

PURPOSE.: Chronic Horner syndrome is a rare clinical condition, the etiology of which often remains undiscovered. A patient is presented with an 8-year history of Horner syndrome who was diagnosed with multiple cervical artery dissections. CASE REPORT.: A 42-year-old woman presented to our emergency department with a severe occipital headache ...

Migraine and metabolic syndrome are highly prevalent and costly conditions. The two conditions coexist, but it is unclear what relationship may exist between the two processes. Metabolic syndrome involves a number of findings, including insulin resistance, systemic hypertension, obesity, a proinflammatory state, and a prothrombotic state. Only one study addresses ...

We use two oriental medical techniques in headache management. One is topological microstimulation, and the other is acupuncture point BL10 (Tianzhu) block. 1. Topological microstimulation The topological microstimulation apparatus delivers programmed fluctuating electrical signals to electrodes placed on the distal portion of the limbs, where meridians are concentrated. Topological microstimulation ...

The Hedgehog signaling pathway has been identified as fundamentally important to normal embryonic development in living organisms ranging from fruit flies to mammals. Postdevelopmentally, it remains active in hair and skin cells. Abnormal activation of components of the Hedgehog pathway-specifically, resulting from mutations in the Patched 1 gene-is associated with ...

The prognosis of parathyroid carcinoma varies significantly between numerous studies. Therefore, many attempts have been made to grade the degree of parathyroid carcinoma, and recently, classifying parathyroid carcinomas into either minimally invasive or widely invasive carcinoma- similar to follicular carcinoma of the thyroid- has led to a more reliable prediction ...

We report the first case of adrenocortical carcinoma secreting cortisol (Cushing's syndrome) and aldosterone (Conn's syndrome) with extensive distant metastasis at the time of diagnosis. A 72-year-old male with exertional dyspnea sought evaluation at our institution. The pattern of tumor spread (lung, pleura, bone and adrenal gland) and respiratory symptoms ...

We report the case of a patient with metastatic renal cell carcinoma with Xp11.2 translocation/transcription factor E3 (TFE3) gene fusion who had presented with sunitinib-induced nephrotic syndrome in association with favorable and durable treatment response. The nephrotic syndrome was managed successfully by discontinuing sunitinib and symptomatic treatment. The 27-year-old female ...

Two types of gynecologic tumors are commonly described in the Turner syndrome, the first one is gonadoblastoma, which occurs in patients with Y chromosome abnormalities, and the second one is endometrial carcinoma which is mostly related with exogenous estrogen usage. Here, we describe an extremely rare case of squamous cell ...

Polymyositis as a paraneoplastic syndrome associated with hepatocellular carcinoma is quite rare; only a few cases have been reported. We report a case of a 50-year-old female who presented with subacute quadriparesis, neck muscle weakness, elevated creatinine phosphokinase, a myogenic pattern on the EMG and was diagnosed as having polymyositis, ...

Nevoid basal cell carcinoma syndrome (NBCCS) is an uncommon autosomal dominant inherited disorder with high penetrance and variable expressivity. It affects multiple organ systems, including the stomatological, skeletal, skin, eye, reproductive, and central nervous systems. It is caused by mutations in the patched tumor suppressor gene, PTCHI, located in the ...

INTRODUCTION: Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder which is characterized by the presence of multiple basal cell carcinomas, maxillary keratocysts, and musculoskeletal anomalies. CASE REPORT: We present a case of a patient suffering from Gorlin-Goltz syndrome who developed an intraosseous ...

Stickler syndrome, also known as hereditary progressive arthro-ophthalmopathy, is an inherited progressive disorder of the collagen connective tissues. Manifestations include short-sightedness, cataracts, retinal problems leading to retinal detachment and possible blindness. This is principally the case among individuals with type 1 Stickler Syndrome. It is the most commonly identified inherited ...

Gorlin syndrome is an autosomal dominantly inherited disorder that results in numerous basal cell carcinomas as well as a number of other facial and skeletal findings. We present a patient with many classic features and review some of the treatment options available for these patients.

Small-cell carcinoma (SCC) of the esophagus is rare, and its clinical characteristics remain poorly understood. A 54-year-old man was given a diagnosis of esophageal SCC and underwent esophagectomy. Four months after surgery, he was admitted to our hospital because of rapidly developing hyponatremia. Syndrome of Inappropriate Secretion of Antidiuretic Hormone ...

A Chinese adolescent girl presented with secondary amenorrhea. During follow-up, she gradually developed Cushingoid features and virilization. After a series of endocrine investigations, including urinary steroid profiling, a diagnosis of adrenocortical carcinoma was made. The treatment and prognosis of the disease are discussed.

Nephrotic syndrome, after administration of tyrosine kinase inhibitors, is uncommon and not well known. A 62-year-old male, who had experienced a left nephrectomy due to a traffic accident 38 years ago, underwent a partial nephrectomy for right renal cell carcinoma (RCC). Histologically, the tumor was a clear cell RCC. Two ...

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatological conditions that are characterized by mucositis, epidermal detachment and erosions. The underlying etiology in SJS and TEN is almost invariably secondary to drugs. Rarely, other causes such as systemic lupus erythematosus (SLE), infections and vaccinations have been implicated. This ...

The Pierre-Robin Syndrome (PRS) is a rare congenital abnormality, with an approximately 1/30,000 estimated rate, characterized by the presence of the combination of mandibular hypoplasia (micrognathia or small jaw), glossoptosis (retrusion of the tongue into the pharyngeal airway) and, often, a posterior cleft of the secondary palate. It may be ...

A 27-year-old Hispanic female was admitted to hospital with fever, a sudden marked decrease in vision, and multi-organ failure shortly after preterm delivery by cesarean section for eclampsia. Her past history was significant for a spontaneous first trimester abortion and one live birth complicated by intrauterine growth retardation. She was ...

A 4.5-year-old spayed female Great Pyrenees with hypothyroidism and hypoadrenocorticism had a slightly enlarged pituitary gland and bilaterally atrophic adrenal and thyroid glands. Lymphocytic adenohypophysitis and adrenalitis were found in which B lymphocytes and plasma cells dominated the adenohypophysitis but T cells dominated the adrenalitis. The thyroid gland had extensive ...

Ocular hypertension caused by endogenous Cushing syndrome from an ectopic adrenocorticotropic hormone-producing tumor is rare. We report an 11-year-old boy who presented with intraocular pressures (IOPs) of 50 mm Hg in both eyes. Surgical resection of the tumor was performed with subsequent normalization of serum cortisol and IOP levels.

Malignancies are usually preceded by the presence of various paraneoplastic syndromes (PNS), which could be the indirect and/or remote effects of the metabolites produced by neoplastic cells. PNS manifested by oropharyngeal squamous cell carcinomas, which is the most common head and neck malignancy, are highlighted in this review. Knowledge of ...

Spontaneous Cushing's syndrome is well known but unusual clinical disorder. Many of the clinical features (central weight gain, glucose intolerance, hypertension, muscle weakness) are seen in other common conditions. Recognition of patients with multiple features, features unusual for their age (i.e. early onset osteoporosis or hypertension), patients with features more ...

We present an atypical case of Cushing's syndrome caused by ectopic adrenocorticotropic hormone (ACTH) secretion in a patient with a metastasised adenocarcinoma of the oesophagus. After chemotherapy and surgery the patient developed generalised oedema, hyperpigmentation and dysphagia. Laboratory tests showed hypokalaemia, normal urinary potassium, increased 24-hour urinary free cortisol excretion ...

Inferior petrosal sinus sampling (IPSS) of ACTH with CRH stimulation helps distinguish pituitary ACTH-dependent Cushing's syndrome from the ectopic ACTH syndrome (EAS). The usefulness of the paradoxical response of other pituitary hormones including prolactin to CRH remains controversial. Data from 33 IPSS procedures carried out at the Walton Centre for ...

Adrenocortical tumors are the most common cause of endogenous Cushing syndrome in infancy and early childhood. Cushing syndrome resulting from ectopic adrenocorticotrophic hormone (ACTH) producing tumor has been infrequently reported in adults. Cortisol secreting pheochromocytoma is rarely reported in literature. We report an eleven month old child presenting to us ...

Pituitary apoplexy is a rare but potentially life-threatening clinical syndrome caused by the sudden enlargement of pituitary adenoma secondary to infarction and/or hemorrhage. It may be the first presentation of previously undiagnosed pituitary adenoma. Although various precipitating factors of pituitary apoplexy are indicated, the pathogenesis remains unknown. In this report, ...

In the past 5 to 10 years, much interest has arisen in the syndrome of occult hyperadrenocorticism. Patients with occult hyperadrenocorticism purportedly have many clinical signs and routine laboratory abnormalities suggestive of the presence of typical hyperadrenocorticism, or Cushing's syndrome (ie, hypercortisolism either due to a pituitary or adrenal tumor). ...

Cushing's syndrome can be caused by adrenocorticotropic hormone-secreting solid tumors. We report a rare case of an ileal endocrine carcinoma that produced ACTH and induced hypercortisolism. A now 47-year-old man presented at age 41 with weight gain, tremor, perspiration, and general fatigue. Laboratory testing showed hypercortisolism and diabetes mellitus. Further ...