Journal of the neurological sciences
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Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare, fatal, neoplastic condition of infiltrating glial cells into the meninges without evidence of primary tumor in the brain or spinal cord parenchyma. Primary diffuse leptomeningeal gliomatosis often presents with symptoms and physical findings of chronic inflammatory meningitis and raised intracranial pressure, and lacks specific clinical, radiologic, and diagnostic criteria. We report a case of PDLG diagnosed post-mortem, highlighting the diagnostic difficulty in identifying PDLG as the cause of chronic meningitis, even when a neoplastic etiology is suspected. Because multiple cytologies and even a leptomeningeal biopsy did not reveal the diagnosis ante-mortem, we emphasize the consideration of multi-site or repeat leptomeningeal biopsy when a persistent inflammatory infiltrate is found and neurological symptoms are progressive.
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NNZ-2566: a Gly-Pro-Glu analogue with neuroprotective efficacy in a rat model of acute focal stroke.
The N-terminal cleavage product of human insulin-like growth factor-1 (IGF-1) in the brain is the tripeptide molecule Glypromate (Gly-Pro-Glu). Glypromate has demonstrated neuroprotective effects in numerous in vitro and in vivo models of brain injury and is in clinical trials for the prevention of cognitive impairment following cardiac surgery. NNZ-2566 is a structural analogue of Glypromate, resulting from alpha-methylation of the proline moiety, which has improved the elimination half-life and oral bioavailability over the parent peptide. ⋯ Neuroprotective efficacy in the MCAO model was also observed following oral administration of the drug (30-60 mg/kg), when formulated as a microemulsion. In vitro, NNZ-2566 significantly attenuates apoptotic cell death in primary striatal cultures, suggesting attenuation of apoptosis is one mechanism of action underlying its neuroprotective effects. NNZ-2566 is currently in clinical trials for the treatment of cognitive deficits following traumatic brain injury, and these data further support the development of the drug as a neuroprotective agent for acute brain injury.
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Comparative Study
FHM3 in familial hemiplegic migraine is more resistant to mutation than FHM1 and FHM2.
Familial hemiplegic migraine (FHM) is a rare subtype of migraine with aura and transient hemiplegia. CACNA1A (FHM1) gene, the ATP1A2 (FHM2) and the SCN1A (FHM3) are reported for their correlation to FHM. ⋯ In this work, the author can identify many mutant prone positions within the studied FHM. Of interest, the author detected that FHM3 is a high resistant molecule when compared to FHM1 and FHM2.
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Spinal cord involvement associated with severe copper deficiency has been reported in the last 8 years. Copper deficiency may produce an ataxic myelopathy. Clinical and neuroimaging findings are similar to the subacute combined degeneration seen in patients with vitamin B12 deficiency. ⋯ Clinical status and neuroimaging improved after copper replacement therapy. Sensory neurons of dorsal root ganglia may be the most sensitive nervous pathway. In this case the early copper treatment allowed to improve neurologic lesions and to prevent further involvements.
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Review Case Reports
Is there need to search for alternatives to indomethacin for hemicrania continua? Case reports and a review.
Hemicrania continua (HC) is a daily continuous unilateral headache of moderate intensity with super imposed exacerbations of more severe pain accompanied by migrainous and cranial autonomic features. Response to indomethacin is an essential feature in the IHS diagnostic criteria. However, indomethacin is associated with a number of side effects. ⋯ Various drugs have been tried as alternatives to indomethacin in the patients intolerant to indomethacin. We report two cases of HC responsive to topiramate and review the available alternatives for the patients of HC. We also discuss the side effects of indomethacin in the various headache disorders and other painful conditions, and suggest the need for trial of other drugs for the patients of HC.