Epilepsies intractable epilepsy

That the hippocampus is important for memory is generally accepted. This is not because it is a site of major degeneration in AzD, that finding can only be used to account for the memory loss if memory is known to be dependent on the hippocampus, but because lesions of that region are known to impair memory. Case reports in the medical literature are rightly mistrusted but few people have felt inclined to disregard the evidence presented by one 27-year-old male mechanic who underwent bilateral hippocampal removal for intractable epilepsy in Montreal in 1953. While that condition was improved the operation has not been repeated because memory loss was almost total, so while he appeared to behave reasonably normally (and still does), he cannot remember where he lives, what he has just eaten or the person he met a few minutes previously. 

Tishler DM, Weinberg KI, Hinton DR, Barbaro N, Annett GM, Raffol C. MDR1 gene expression in brain of patients with medically intractable epilepsy. Epilepsia 1995 36 1-6. 

Uvebrant, P., and Bauziene, R. (1994) Intractable epilepsy in children the efficacy of lamotrigine treatment, including non-seizure-related benefits. Neuropediatrics 25 284—289. 

While there seems to be little in common between intractable epilepsy and cancer, researchers in each field have focused on a common mechanism underlying multidrug resistance a cellular pump called P-glycoprotein (Pgp). Pgp protects cells from toxic substances by actively excreting the toxic agent. The pumps reside in tissues that are extensively exposed to toxic material liver, lungs, kidney, intestine, placenta, and blood-brain barrier. How-... 

GAMT deficiency has been known for more than 10 years now and several affected patients have been reported [6]. The severity of clinical symptoms varies widely. Affected patients show developmental delay with absence of active speech and, in older patients, autism with self injury. More severely affected patients present with severe extrapyramidal symptoms and intractable epilepsy. Biochemical diagnosis can be made by analysis of GA in the plasma or urine. 

Propofol infusion syndrome might be precipitated by a combination of prolonged propofol infusion and carbohydrate intake insufficient to suppress fat metabolism. Support for this hypothesis has come from a case report of a child with catastrophic epilepsy who developed fatal propofol infusion syndrome after a ketogenic diet was introduced in an attempt to control severe intractable epilepsy (962). 

Secondary Epileptogenesis, Kindling, and Intractable Epilepsy A Reappraisal from the Perspective of Neuronal Plasticity Thomas P. Sutula... 

Between January 1983 and March 1994, 302 patients with intractable epilepsy were entered into a nitrazepam compassionate plea protocol (2). Nitrazepam increased the risk of death, especially in young patients with intractable epilepsy. The authors suggested that nitrazepam should be used with caution in young children with intractable epilepsy, especially if they have difficulties in swallowing, aspiration, pneumonia, gastroesophageal reflux, or a combination of these. 

Rintahaka PJ, Nakagawa JA, Shewmon DA, Kyyronen P, Shields WD. Incidence of death in patients with intractable epilepsy during nitrazepam treatment. Epilepsia 1999 40(4) 492-6. 

Angelman s syndrome, a genetic disorder that involves a defect in the DNA coding for subunits of the GABA-A receptor, is often associated with intractable epilepsy. Topiramate was effective in five children with Angelman s syndrome and epilepsy (645). One patient had transient insomnia and one had akathisia and insomnia that persisted until topiramate was withdrawn. 

Sills, M. A Forsythe, W, L., Haidukewych, D., MacDonald, A-, and Robinson, M. (1986). The medium-chain iriglyccride diet and intractable epilepsy. Arch- Dts. Child. 61,1168-1172. 

French J, Smith M, Faught E, Brown L. Practice advisory the use of felbamate in the treatment of patients with intractable epilepsy. Epilepsia 1999 40 803-8. 

Sigler M, Strassburg HM, Boenigk HE. Effective and safe but forgotten methsuximide in intractable epilepsies in childhood. Seizure 2001 10(2) 120-4. 

SED-13,1311) (1). Protirelin has neurotransmitter properties and has been used to treat a variety of neurological disorders, including intractable epilepsy (2). Some experiments have also been performed to evaluate its effects in mental disorders. 

Kubota M, Nishi-Nagase M, Sakakihara Y, Noma S, Nakamoto M, Kawaguchi H, Yanagisawa M. Zonisamide-induced urinary lithiasis in patients with intractable epilepsy. Brain Dev 2000 22(4) 230-3. 

Multiple surgical biopsies (0.02-0.1 g) were obtained during resections from brain tumours and from the active site and underlying white matter of patients with intractable epilepsies. Proton MR spectra were obtained on a Bruker AM 8.5 T spectrometer at 37°C. The data set consisted of the H MR spectra of tissue specimens from meningiomas (M-95 24 cases) and astrocytomas (A-74 26 cases), and intractable epilepsy as the control (E-37 8 cases). 

M. Sillanpaa in S. I. Johannessen, L. Gram, M Sillanpaa, and T. Thomson, Eds., Intractable Epilepsy, Wrightson Biomedical Publishing, Petersfield, pp. 13-25. 

Smith DF, Baker GA, Dewey M, et al. Seizure frequency, patient-perceived seizure severity and the psychosocial consequences of intractable epilepsy. Epilepsy Res 1991 9 231-41. 

Similar to this patient. Summers et al. describe the case of a 24-year-old woman suffering from intractable epilepsy, who also profited from verapamil coadministration [52]. These findings suggest that despite the fact that most AEDs seem to be poor ABCBl substrates, verapamil can help increase AED concentration in epileptogenic tissues and thus can alleviate RE. It has to be noted, however, that verapamil is not a specific inhibitor of ABCBl and may rather alter the properties of the BBB (i.e., tight junctions) by blocking Ca -channels, thus enabling paracellular influx of AEDs into the brain. The exact mechanism of how verapamil enables AED treatment of RE remains to be elucidated. 

Aronica, E. et al. (2003) Expression and cellular distribution of multidrug transporter proteins in two major causes of medically intractable epilepsy focal cortical dysplasia and glioneuronal tumors. Neuroscience, 118 (2), 417-429. 

Rarribeck, B. et al. (2006) Comparison of brain extracellular fluid, brain tissue, cerebrospinal fluid, and serum concentrations of antiepileptic drugs measured intraoperatively in patients with intractable epilepsy. Epilepsia, 47 (4), 681-694. 

Schwartz RM, Boyes 8, Aynsley-Green A. Metabolic effects of three ketogenic diets in the treatment of intractable epilepsy. Dev Med Child Neurol 1989 31 152-160. 

Sirven J, Whedon B, Caplan D, Liporace J. Glosser I ), O Dwyer J, et al. The ketogenic diet for intractable epilepsy in adults preliminary results. Epilepsia 1999 40 1721-1726. 

Intractable epilepsy. In patients who have confirmed IgG deficiency, IVIG may be useful. For certain syndromes, such as West or Lennox-Gastaut syndrome, IVIG may be considered. ... 

Prater JL, Prayson RA, Morris 111 HH, et al. Surgical pathologic findings of extratemporal-based intractable epilepsy A study of 133 consecutive reactions. Arch Pathol Lab Med. 2000 124 545-549. 

---