CANOMAD syndrome with respiratory failure.

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient's disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients' quality of life.

[Preliminary experience with controlled lumbar drainage in medically refractory intracranial hypertension]. / Spinalis liquorlebocsátással szerzett kezdeti tapasztalatok koponyasérülést követo befolyásolhatatlan intracranialis nyomásfokozódás kezelésében.

INTRODUCTION: The contribution of brain edema to brain swelling in cases of traumatic brain injury remains a critical problem. In head injury, the swelling and eventual rise in intracranial pressure is a frequent cause of death, and in survivors the poor prognosis with sustained elevation of ICP has been well documented. OBJECTIVE: The objective this study was to evaluate the effect of controlled lumbar cerebrospinal fluid drainage in adult patients with refractory intracranial hypertension following severe brain injury. METHOD: The study involved 10 head injured patients (GCS < or = 8) with medically refractory intracranial hypertension. Aggressive treatment included the repeated steps of the Brain Trauma Foundation's guidelines, barbiturate coma and in many cases decompressive craniectomy as well. After institution of a lumbar drain, cerebrospinal fluid drainage was maintained under control of intracranial pressure (ICP) and neurological status. ICP and cerebral perfusion pressure before and after initiation of lumbar cerebrospinal fluid drainage and related complications were documented. RESULTS: All patients demonstrated an immediate decrease of ICP (from 30.6 +/- 4.7 mm Hg to 11.5 +/- 3.9 mm Hg, mean +/- SD) and a concomitant increase of cerebral perfusion pressure. In seven patients the decrease of ICP was long lasting and 5 of them had a favourable outcome. Two patients survived with a severe permanent neurologic deficit and only three patients died because of the progressive brain edema, which developed despite of the maximum therapy. CONCLUSION: In conclusion we may consider, that controlled lumbar cerebrospinal fluid drainage is a potentially useful treatment in cases of severe traumatic brain injury when maximal medical therapy and ventricular cerebrospinal fluid evacuation have failed to control high intracranial hypertension. The danger of herniation is minimized by considering lumbar drainage in the presence of discernible basilar cisterns only.