Biomedical subjects

R Hessler

Publications and source records attributed to R Hessler.

6 recordsLinked to original sources

PubMed2001

Extra-axial cavernous hemangioma: two case reports.

Two patients with extra-axial cavernous hemangioma who presented with headache and oculovisual disturbances were investigated with computed tomography and magnetic resonance imaging. The lesions masqueraded as basal meningioma, but this diagnosis was not supported by magnetic resonance spectroscopy in one patient. Cerebral angiography with embolization was indicated in one patient, but embolization was not justified in the other. Both patients underwent a pterional craniotomy. The lesions were extradural and highly vascular, necessitating excessive transfusion in one patient in whom gross total resection was achieved, and precluding satisfactory removal in the other. There was no mortality. Transient ophthalmoplegia, the only complication in one patient, was due to surgical manipulation of the cavernous sinus; it resolved progressively over 3 months. Extra-axial skull base cavernous hemangiomas are distinct entities with clinical and radiological characteristics that differ from those of intraparenchymal cavernous malformations. They can mimic meningiomas or pituitary tumors. In some cases, magnetic resonance spectroscopy may narrow the differential diagnoses. Surgical resection remains the treatment of choice, facilitated by preoperative embolization to reduce intraoperative bleeding and by the application of the principles of skull base surgery. Fractionated radiotherapy is an alternative in partial or difficult resections and in high-risk and elderly patients.

Case Reports↗

PubMed2000

ACTH-producing pituitary cancer: experience at the King Faisal Specialist Hospital & Research Centre.

Pituitary gland is an uncommon site of a primary cancer. Of more than 600 cases of pituitary tumors seen at the KFSH&RC between 1975 to 1998 only 3 patients had primary pituitary cancer. We have previously reported a case of pituitary fibrosarcoma arising as a rare complication of external radiotherapy (ERT) for GH-secreting pituitary adenoma (PA) [1]. We report now 2 cases of ACTH-producing primary pituitary carcinoma (ACTH-PPC); their follow-up data provide information on the natural history of this cancer. Patient #1; a 46 year old lady with Cushing's disease (CD) presented with an enlarged right cervical lymph node (LN) 2 years after having undergone a partial hypophysectomy through transsphenoidal surgery (PHYPX/TSS) and ERT for an invasive pituitary tumor. Patient #2; a 26 year old man presented with CD and underwent bilateral adrenalectomy (ADx) and pituitary ERT. Thirty-nine months later he developed Nelson's syndrome and a PHYPX/TSS was performed. Incidentally discovered hepatic metastases in this patient and an excisional biopsy of the LN in patient #1 showed histological features very similar to the pituitary tumor, and they stained strongly positive for ACTH. Perinuclear spherical hyalinized cytoplasmic inclusions were seen in the LN biopsy that corresponded to bundles of type 1 microfilaments (specific for pituitary ACTH-producing cells) seen by electron microscopy. A whole body 18-Fluoro-2-Deoxy-D-Glucose positron emission (FDG-PET) scanning, showed an intense uptake in the neck mass. A trial of octreotide did not change the exceedingly high levels of ACTH in patient #2, further supporting the diagnosis of ACTH-PPC. The clinical course of 102 months prior to his demise showed continued progression of the primary and the metastatic tumor. Patient #1, is alive at 15 months follow-up; hypercortisolemia is controlled using ketoconazole. ACTH-PPC should be entertained in a patient with CD presenting with persistent cervical lymphadenopathy. The clinical course in our patients suggests that the emergence of PC may involve a proliferative continuum from a pre-existing PA to an invasive tumor, culminating in a carcinoma. Adjunctive events such as ERT/ADx may predispose to the evolution of PC in genetically susceptible individuals. Because ERT is an effective treatment for PA its use will continue; it is important to be aware of the possible complication of primary pituitary carcinoma.

Adrenocorticotropic Hormone↗

PubMed1998

Miyoshi myopathy in Saudi Arabia: clinical, electrophysiological, histopathological and radiological features.

Miyoshi myopathy (MM) is a rare autosomal recessive distal myopathy linked to chromosome 2p12-14 that has not been described in Saudi Arabia. A Saudi family with five siblings aged 3-25 years, an unrelated 18-year-old woman and a 40-year-old man with MM were identified. All patients underwent a neurological examination, serum chemistry, electromyography and MRI of the legs. Four patients underwent a muscle biopsy that was processed for routine enzyme histochemistry and immunocytochemical analyses for dystrophin and adhalin (alpha-sarcoglycan). The two sporadic and two familial cases showed classic findings of MM, including early adult onset, preferential involvement of gastrocnemius muscles, markedly elevated serum creatine kinase levels and dystrophic-appearing muscle without vacuoles. Magnetic resonance imaging revealed selective involvement of the posterior compartment muscles and myoedema by STIR sequences. The remaining three familial cases had elevated serum creatine kinase levels and two also had early myopathic findings by EMG suggestive of MM.

Adolescent↗

PubMed1993

The 39-kilodalton outer membrane protein of Proteus mirabilis is an OmpA protein and mitogen for murine B lymphocytes.

Partial amino acid sequence analysis of a major outer membrane protein of Proteus mirabilis (39-kDa protein) indicates that it is an OmpA protein. The mitogenic activities of the 39-kDa protein for murine lymphocytes were also investigated with T lymphocytes isolated by passing spleen cells over columns of nylon wool fiber and B lymphocytes obtained by treating spleen cells with monoclonal antibodies to Thy1 plus complement. The 39-kDa protein showed little activity in stimulating T cells to proliferate but was strongly mitogenic for B cells.

Amino Acid Sequence↗

PubMed1988

Emergency flexible fiberoptic nasotracheal intubation: a report of 60 cases.

We describe our experience with 60 consecutive intubations using flexible fiberoptic nasotracheal technique in the emergency setting. Fifty-seven of the procedures were carried out by two emergency physicians initially trained on intubation manikins. A learning curve is constructed demonstrating that time to intubation is decreased after nine or ten intubations. Complications are reviewed. Bleeding occurred in 22% of patients; the technique failed in 13%. Failure to intubate with fiberoptic technique was associated with specific problems such as bleeding, tumor, or agitation. Our results demonstrate both the limitations and special use of flexible fiberoptic technique.

Airway Obstruction↗

PubMedDate not supplied

Retinoblastoma as a congenital primary intracranial tumor.

This report describes a rare case of retinoblastoma presenting as a congenital primary intracranial tumor. A 7-day-old infant presented with generalized seizures. He had a family history of retinoblastoma. Computed tomography of the brain revealed a midline suprasellar tumor without evidence of retinal tumors. Subtotal resection of the tumor was performed. Histopathologic findings were diagnostic of retinoblastoma. The patient sustained profound ischemic brain damage and was not given definitive further therapy. He died days after the diagnosis was made. Retinoblastoma can present as an isolated ectopic intracranial tumor without associated retinal tumors.

Brain Neoplasms↗