Polyglandular Autoimmune Syndrome Type III with Primary Hypoparathyroidism

Polyglandular autoimmune syndrome is defined as multiple endocrine gland insufficiencies accompanied by autoimmune diseases of the endocrine and nonendocrine system. After Schmidt introduced a case of nontuberculosis adrenal gland dysfunction with thyroiditis in 1926, Neufeld defined polyglandular autoimmune syndrome by I, II, and III subtypes in 1980 by their presentation of occurrence age, heredity methods, relationship with human leukocyte antigen, and accompanying diseases. We report a case of a 32-year-old female with polyglandular autoimmune syndrome III accompanied by type 1 diabetes mellitus that was treated with insulin (36 units per day) for 11 years. She had insulin deficiency and Hashimoto thyroiditis as an autoimmune disorder. In addition, she had several features similar to Albright's hereditary osteodystrophy including short stature, truncal obesity, round face, short neck, low intelligence (full IQ 84), and decreased memory. Although Albright's hereditary osteodystrophy is morphological evidence of pseudohypoparathyroidism or pseudopseudohypoparathyroidism, she had primary hypoparathyroidism on laboratory results. Here, we report a case of polyglandular autoimmune syndrome III with type 1 diabetes mellitus, autoimmune thyroiditis, and primary hypoparathyroidism, accompanied by clinical features similar to Albright's hereditary osteodystrophy.

Isolated Oculomotor Nerve Palsy Following Minor Head Trauma : Case Illustration and Literature Review

Isolated oculomotor nerve palsy (ONP) attributable to mild closed head trauma is a distinct rarity. Its diagnosis places high demands on the radiologist and the clinician. The authors describe this condition in a 36-year-old woman who slipped while walking and struck her face. Initial computed tomography did not reveal any causative cerebral and vascular lesions or orbital and cranial fractures. Enhancement and swelling of the cisternal segment of the oculomotor nerve was seen during the subacute phase on thin-sectioned contrast-enhanced magnetic resonance images. The current case received corticosteroid therapy, and then recovered fully in 13 months after injury. Possible mechanism of ONP from minor head injury is proposed and previous reports in the literature are reviewed.

A Case of Central Cord Syndrome Related Status Epilepticus: A Case Report

Central cord syndrome (CCS) is extremely rare as a direct consequence of generalized epileptic seizure. CCS is associated with hyperextension of the spinal cord and has characteristic radiologic findings including posterior ligamentous injury and prevertebral hyperintensity following magnetic resonance imaging (MRI). We experienced the case of a 25-year-old man who suffered CCS after status epilepticus. Cervical spinal MRI revealed high signal intensity at the C1 level but with no signal or structural changes in other sites. After rehabilitation management, the patient significantly improved on the ASIA (American Spinal Injury Association) motor scale and bladder function. We proposed that epilepsy related CCS may be caused by muscle contractions during generalized seizure, which can induce traction injury of the spinal cord or relative narrowing of spinal canal via transient herniated nucleus pulposus or transient subluxation of vertebra. We also suggest CCS without radiologic findings of trauma has good prognosis compared with other CCS.

The Effect of Octreotide LAR on GH and TSH Co-Secreting Pituitary Adenoma

Growth hormone (GH) and thyroid stimulating hormone (TSH)-secreting pituitary adenomas are very rare and they account for only 0.5% for all pituitary adenomas. These adenomas are usually treated with surgery, but this surgery is not easy because the tumor is usually huge and invasive. We reported here on a case of a GH-TSH-secreting adenoma in a 23-year-old male patient who was initially treated with octreotide LAR. He presented with symptoms of headache, palpitation and a visual defect that he had for the 3 months. He had hypertrophy of the frontal bone and enlargement of both the hands and feet. The visual field test showed bitemporal hemianopsia. The laboratory examinations showed high serum levels of free T4, TSH and free alpha-subunit. Additionally, the serum levels of GH and insulin-like growth factor-I (IGF-I) were increased. GH was not suppressed below 1microg/L by an oral 75g glucose loading test, and TSH was not stimulated by thyrotropin-releasing hormone (TRH). Because sellar MRI showed invasive macroadenoma encasing the vessels, we initially tried octreotide LAR for treatment. A year later, the IGF-I and thyroid function tests were normalized and the size of the tumor was reduced with cystic change. The symptoms of palpitation and headache were improved without a change of the visual field defect.

Churg-Strauss Syndrome Presenting as Transverse Myelitis / 대한류마티스학회지

Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare disorder characterized by the presence of asthma, eosinophilia and small-to-medium-sized vessels vasculitis. The peripheral nervous system is frequently involved in CSS, but central nervous system (CNS) involvement is rare. Furthermore, transverse myelitis (TM) as a presenting symptom in patients with CSS is extremely rare. We report here on a 60-year-old female who presented peripheral eosinophilia, lung eosinophilic infiltration, lung vasculitis, and TM. She was diagnosed as CSS based on clinical manifestation, pathologic findings, and the use of magnetic resonance imaging (MRI).