J Neurol (2007) 254:1012– 1017 DOI 10.1007/s00415-006-0479-2
Marco Spinazzi Franca De Lazzari Bruno Tavolato Corrado Angelini Renzo Manara Mario Armani
Received: 26 June 2006 Received in revised form: 25 September 2006 Accepted: 9 October 2006 Published online: 6 April 2007
M. Spinazzi (&) Æ B. Tavolato C. Angelini Æ M. Armani Second Neurological Unit Dept. of Neurosciences University of Padova Ospedale S. Antonio, Via Facciolati 71 35100 Padova, Italy Tel.: +39-049/8215309 Fax: +39-049/8078341 E-Mail:
[email protected] F. De Lazzari Gastroenterology Unit Ospedale S. Antonio, Via Facciolati 71 35100 Padova, Italy R. Manara Neuroradiological Unit General Hospital of Padova Via Giustiniani 2 35100 Padova, Italy
ORIGINAL COMMUNICATION
Myelo-optico-neuropathy in copper deficiency occurring after partial gastrectomy Do small bowel bacterial overgrowth syndrome and occult zinc ingestion tip the balance?
j Abstract Acquired copper defi-
ciency has recently been recognized as a cause of myeloneuropathy mimicking subacute combined degeneration due to vitamin B-12 deficiency. A remote history of gastric surgery is frequently associated with this syndrome. However, the very limited prevalence of severe copper deficiency in patients with a history of gastric surgery suggests that additional contributing factors are likely to be involved. We describe a patient with copper deficiency and a previous Billroth II partial gastrectomy for gastric carcinoma, presenting with severe myelo-optico-neuropathy, demyelinating lesions of the brain, and subjective hyposmia. An abnormal glucose breath test also revealed small bowel bacterial overgrowth
Introduction Copper is a ubiquitous trace element whose deficiency can affect different target organs as bone marrow, the cardiovascular system [1], and the central and peripheral nervous systems. Since the first report by Schleper et al. [2] in 2001, a limited, but increasing number of patients with copper deficiency myeloneuropathy have been described [3–14]. Neurologically this syndrome closely mimics subacute combined degeneration due to vit B-12 deficiency, with characteristic involvement of the dorsal and lateral columns of the spinal cord, optic nerves,
syndrome. Copper replacement therapy associated with antibiotic therapy was effective in preventing further neurological damage and in obtaining mild improvement. We propose that copper status should be evaluated in all patients presenting with unexplained noninflammatory myeloneuropathy. Small bowel bacterial overgrowth syndrome should be investigated as a cause of generalized malabsorption and a possible contributing factor to copper deficiency after gastric surgery, as should occult zinc ingestion.
j Key words copper deficiency Æ spastic paraparesis Æ optic neuropathy Æ malabsorption neuropathy
peripheral nerves, and cerebral white matter. Copper loss has also been advocated as a possible cause of olfactory dysfunction [15].
Case report A 62-year-old woman was admitted to our clinic in September 2003 for subacute onset of progressive gait ataxia, ascending paresthesias in the lower extremities, hyposmia, hypogeusia, blurred vision, and urinary incontinence, which began after significant, unexplained weight loss, ()15% of the total body weight over six months). Her past medical history was remarkable for Billroth II partial gastrectomy performed for a muciparous gastric adenocarcinoma 12 years before presentation, well-controlled ischemic coronary
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Fig. 1 FSE T2 cervical spine MRI, sagittal (A) and axial views (B), showing a longitudinal lesion (arrows) in the central posterior spinal cord
artery disease, and microcytic anaemia. A brain and a complete spine MRI performed at the beginning of the disease were normal. Neurological examination showed severe spastic-ataxic paraparesis, gait was wide-based and dependent on assistance, and a positive Romberg sign was present. The patient had mild distal muscle weakness and diffuse muscle wasting. Pyramidal involvement was indicated by very brisk tendon reflexes with extensor plantar reflex on the right side, equivocal on the left side. A profound dorsal column dysfunction was present with severely decreased vibratory sensation up to the anterior superior iliac spine, impaired position sense in the toes and pseudo-athetosis. Pin prick and touch sensations gradually decreased from D12 to the toes. Mild bilateral optic pallor was revealed by fundoscopy. The patient reported concomitant severe hyposmia and hypogeusia, for which she underwent a thorough ORL exam, which was not significant. An EMG revealed a diffuse reduction in compound muscle action potentials in the four limbs, with fibrillation potentials and normal conduction velocities consistent with diffuse axonal motor neuropathy. Motor evoked potentials showed reduced amplitude and central motor conduction delay to the abductor hallucis muscle bilaterally (27 ms; nv: 10.1–16.3). Sensory evoked potentials showed an undetectable evoked response at lumbar level and mild delay in the upper extremities. Increased latency of visual evoked potentials were recorded bilaterally (P100 121 ms on the left side, 113.5 ms on the right side; nv
