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Biomedical subjects

K C Golnik

Publications and source records attributed to K C Golnik.

27 records · Page 2Linked to original sources

Ophthalmic involvement in myo-neuro-gastrointestinal encephalopathy syndrome.

We studied the clinical, histopathologic, neuroradiologic, biochemical, and genetic profile of a patient with the myo-neuro-gastrointestinal encephalopathy syndrome, a recently described multisystem mitochondriopathy characterized by blepharoptosis and ophthalmoparesis. The patient had severe intestinal pseudo-obstruction and a mixed demyelinating and axonal neuropathy. Abnormal collections of mitochondria in nerve and muscle as well as diffuse white matter disease were present. Cytochrome oxidase activity in muscle mitochondria was reduced. No mitochondrial DNA deletions were detected.

Adult↗

Diagnosis of cavernous sinus arteriovenous fistula by measurement of ocular pulse amplitude.

BACKGROUND: The ocular pulse amplitude (OPA), the difference between the maximum and minimum intraocular pressure (IOP) during the cardiac cycle, has been reported to be elevated in the eye ipsilateral to a cavernous sinus arteriovenous fistula. METHODS: The OPA was measured with a pneumotonometer in three groups of patients. Patients in group 1 had no orbital disease (n = 50), patients in group 2 had either unilateral or asymmetric orbital disease (n = 30), and patients in group 3 had angiographically proven cavernous sinus arteriovenous fistulas (n = 15). RESULTS: Patients in group 3 had a higher OPA than patients in either group 1 (P less than 0.001) or group 2 (P less than 0.001). The difference in OPA between an individual's eyes (delta OPA) also was higher in patients with cavernous sinus arteriovenous fistulas than in patients without fistulas (P less than 0.0001). CONCLUSION: A delta OPA of more than 1.6 mmHg was 100% sensitive and 93% specific in identifying patients with cavernous sinus arteriovenous fistulas. There was no difference in delta OPA between direct and dural cavernous sinus arteriovenous fistulas. Successful transvascular embolization of the cavernous sinus arteriovenous fistula normalized the delta OPA by reducing the OPA on the affected side.

Adult↗

Rate of progression and severity of neuro-ophthalmologic manifestations of cavernous sinus meningiomas.

The progression of neuro-ophthalmologic signs and symptoms caused by cavernous sinus meningiomas (CSMs) was evaluated in 24 patients. Ten patients had primary involvement of the cavernous sinus by meningioma, and 14 patients had extension of a sphenoid ridge meningioma into the cavernous sinus. Eighteen patients were followed after intradural meningioma debulking. Two of these patients underwent conventional radiation therapy after surgery. The other six patients were followed without treatment. Optic neuropathy caused by extension of the CSM was the most frequently (67%) seen manifestation at the beginning of the follow-up period. Proptosis (50%), ocular motor nerve palsies (46%), and trigeminal neuropathy (33%) were also common. During a mean follow-up period of 57 months, 14 patients (58%) had no change in neurologic status, four patients (17%) had improvement in one or more parameters and six patients (25%) worsened. The patients who worsened had progression of preexisting cranial nerve palsies (two patients), developed new cranial neuropathies (three patients), or both (one patient). Patients who worsened had a significantly longer mean follow-up (76 months) than patients who remained stable or improved (47 months) (p = 0.01). Although the signs and symptoms of CSMs may worsen with time, the rate is slow and the degree is mild. These factors are important when considering treatment options.

Journal Article↗

Late recovery of function after oculomotor nerve palsy.

We studied three patients who developed oculomotor nerve paresis from different causes. Each patient improved somewhat over several months, after which there was no further improvement for at least six months. Although the pareses were thought to be stable after the period of no improvement, each patient subsequently had further improvement in both motility and alignment with resolution of diplopia in primary position and in more than one of the cardinal positions of gaze. Patients with oculomotor nerve paresis may improve further after an initial period of improvement followed by several months of stability.

Adenoma↗

Cryptococcal optic neuropathy in the acquired immune deficiency syndrome.

Cryptococcus neoformans infection occurs frequently in patients with the acquired immune deficiency syndrome (AIDS). Cryptococcal meningitis can result in optic neuropathy. Improvement in afferent visual system dysfunction has not been documented. We report three patients with AIDS who developed either unilateral (1) or bilateral (2) afferent visual system dysfunction. The bilaterally affected patients had visual field deficits compatible with chiasmal involvement. All patients had improvement in their vision following appropriate treatment with amphotericin B. Reactivation of cryptococcal infection was heralded by neuro-ophthalmic manifestations in two patients, in spite of maintenance therapy. Despite the poor overall prognosis, AIDS patients with presumed cryptococcal optic neuropathy can benefit from optimal therapy.

Acquired Immunodeficiency Syndrome↗

Anterior ischemic optic neuropathy associated with macrocytic anemia.

A 37-year-old man experienced the acute onset of blurred vision, particularly in the inferior hemifield of his left eye. Neuro-ophthalmic examination showed a left afferent pupillary defect, a left inferior altitudinal visual field deficit, bilateral nerve fiber layer infarcts and hemorrhages, and left optic disc elevation with edema of the nerve fiber bundle. Complete examination and laboratory studies revealed only a severe folate deficiency anemia. This is the first well-documented report of anterior ischemic optic neuropathy associated with anemia in the absence of other systemic abnormalities.

Adult↗

Spectroscopic and kinetics studies of the inhibition of pig kidney diamine oxidase by anions.

The role of copper in pig kidney diamine oxidase has been probed by examining the effects of potential Cu(II) ligands on the spectroscopic and catalytic properties of the enzyme. In the presence of azide and thiocyanate, new absorption bands are evident at 410 nm (epsilon = 6300 M-1 cm-1) and 365 nm (epsilon = 3000 M-1 cm-1), respectively. These bands are assigned as ligand-to-metal charge-transfer transitions, N3-/SCN- leads to Cu(II). One anion/Cu(II) is coordinated in an equitorial position. Anion binding can be completely reversed by dialysis. The equilibrium constants for diamine oxidase-anion complex formation are 134 M-1 (N3-) and 55 M-1 (SCN-). Azide and thiocyanate are linear uncompetitive inhibitors with respect to the amine substrate when O2 is present at saturating concentrations. Taken together, the data are consistent with a functional role for Cu(II) in diamine oxidase catalysis.

Amine Oxidase (Copper-Containing)↗