Clinical and electrodiagnostic findings in copper deficiency myeloneuropathy.

INTRODUCTION: Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency. METHODS: Clinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of copper deficiency. Patients with other potential causes of myelopathy or neuropathy were excluded. RESULTS: The predominant clinical feature in all six patients was a sensory ataxia, resulting in marked gait unsteadiness. Nerve conduction studies and needle EMG were performed in all patients and revealed a mild to moderate distal, axonal, sensorimotor peripheral neuropathy. Median and tibial somatosensory evoked potentials were abnormal in all five patients in which it was performed, showing impaired conduction in central or proximal peripheral somatosensory pathways. CONCLUSIONS: This pattern of electrodiagnostic findings suggests that impairment in somatosensory pathways demonstrated by somatosensory evoked potential testing is the main cause of the sensory ataxia in patients with copper deficiency.

Approach to the evaluation of small fiber peripheral neuropathy and disorders of orthostatic intolerance.

Small fiber peripheral neuropathy is a frequently encountered neurological disorder, which can be difficult to diagnose. In this article, the differential diagnosis of small fiber neuropathy is discussed, along with role of autonomic testing, skin biopsy, and quantitative sensory testing, in establishing a definitive diagnosis of small fiber peripheral neuropathy. Disorders of orthostatic intolerance, including postural orthostatic tachycardia syndrome (POTS), are also discussed, emphasizing diagnostic evaluation and a treatment approach to these disorders.

Copper deficiency myeloneuropathy resembling B12 deficiency: partial resolution of MR imaging findings with copper supplementation.

Copper deficiency has been associated with a clinical syndrome, myeloneuropathy. Radiographic changes resembling B(12) deficiency in the cervical spinal cord have been described. We present a case of copper deficiency myeloneuropathy, with cervical MR imaging findings resembling B(12) deficiency, which partially reversed following copper supplementation. This is, to our knowledge, the first described case of radiographic improvement with copper supplementation.

The effect of oxygen tension on the growth and metabolism of WI-38 cells.

The effect of oxygen tension on cellular growth and metabolism was studied in actively growing WI-38 cells [greater than 90% labeled nuclei (LN)] grown under atmospheres containing 5% CO2 and various combinations of O2 and N2. Cells grown under a partial pressure of oxygen (PO2) of 7.8 +/- 3.5 mm Hg had a significantly slower growth rate, lower saturation densities and higher rates of glucose consumption and lactate production than did cells grown under a PO2 of 44 +/- 7 mm Hg. There were no significant differences in saturation density or the rates of glucose consumption or lactate production between cells grown under PO2 26 +/- 4 mm Hg, 44 +/- 7 mm Hg, or 134 +/- 11 mm Hg. Population doubling time was slightly prolonged at a PO2 of 134 mm Hg compared to a PO2 of 44 mm Hg. Cells grown under a PO2 of 291 +/- 25 mm Hg showed only 20-30% of the growth rate and 10-20% of the saturation density of cells grown under a PO2 of 134 mm Hg. Despite this reduced growth, cells grown under a PO2 of 291 mm Hg consumed four to six times as much glucose and produced four to six times as much lactate per cell as cells grown at a PO2 of 134 mm Hg. Cells grown under a PO2 of 560 +/- 38 mm Hg attached but did not proliferate. This toxic effect of oxygen on cell proliferation was reversible and was not due to an effect of oxygen on the media.