Ettinger a. Neurology 2006;67: 1916-1925. Review paper. Random notes
Gabapentin--
1. aberrant/aggressive behavior noted in children and patients with developmental disorders. 2. Ineffective for BPAD 3. It does help behaviors in institutionalized patients with AD 4. Useful in epileptic patients with anxiety
Lamotrigine-- 1. It does help mood in depressed patients with epilepsy. 2. Effective in controlling impulsive aggression in one study.
Leviteracetam-- 1. Causes depression or anxiety in up to 15 % of those treated, especially those with preexisting disorders that are exacerbated with the drug. 2. Reversible psychotic symptoms occur in children treated. 3. May be ameliorated with pyridoxine. 4. Tested for treatment of mania and social anxiety syndrome
oxcarbazepine-- too limited information to make conclusions.
pregabalin--similar to gabapentin. Useful for epilepsy patients with anxiety.
topiramate-- related to psychomotor slowing. Used in bPAD for weight reducing side effect. Not proven efffective in BPAD. May be effective for anger/aggression in those with borderline personalities. Other side effects in 1-2 % of patients (7 % of kids) are psychotic symptoms, agitation, aggression, hallucinations, auditory and visual, paranoid and mystical delusions. Does not stabilize moods.
tiagabine-- possibly anxiolytic but not a mood stabilizer.
Thursday, December 28, 2006
Fludaribine for MGUS
Niermeijer JMF et al. Neurologic and hematologic response to fludaribine treatment in IgM MGUS polyneuropathy. Neurology 2006:67:2076-2079.
Uncontrolled trial of 16 patients with IgM with progressive IgM MGUS treated with fludaribine.
Patients: 6 were MAG positive, 14 had kappa light chain, 2 had lambda light chain, 3 had axonal neuropathy and 13 had demyelinating neuropathy. 9 used ambulation aids.
10/16 were first treated with cyclophosphamide/prednisone
Uncontrolled trial of 16 patients with IgM with progressive IgM MGUS treated with fludaribine.
Patients: 6 were MAG positive, 14 had kappa light chain, 2 had lambda light chain, 3 had axonal neuropathy and 13 had demyelinating neuropathy. 9 used ambulation aids.
10/16 were first treated with cyclophosphamide/prednisone
4-H syndrome
Timmons et al. Peripheral and central hypomyelination with hypogonadotropic hypogonadism and hypodontia. NIH. Neurology 2006; 67:2066-2069.
Pubertal onset dysmyelinating leukodystrophy. Characteristics: young adults, absent spontaneous puberty, normal till age 7-12, borderline iq's (60s-70s), dysarthria, absent pursuit, upbeat or other nystagmus, normal sensory exam, dysmetria, long tract signs.
Sural nerve biopsies showed membraneous and other debris, loss of major and minor dense lines, increased neurofilaments. Decreased galactocerebrosidase and sphingomyelin. Immunostaining for PO protein showed clefted disruption of myelin sheaths and laminar deposits in circumferential distribution. MAG staining was similar but less definitive. Nerve conductions were normal.
MRI showed supratentorial white matter signal approximated cortex on T1, hyperintense WM on T2 (demyelination) atrophic cerebellum and thin corpus callosum.
Heterogenous Holmes-Adie syndrome has described the combination of cerebellar ataxia and hypogonadotropic hypogonadism.
Pubertal onset dysmyelinating leukodystrophy. Characteristics: young adults, absent spontaneous puberty, normal till age 7-12, borderline iq's (60s-70s), dysarthria, absent pursuit, upbeat or other nystagmus, normal sensory exam, dysmetria, long tract signs.
Sural nerve biopsies showed membraneous and other debris, loss of major and minor dense lines, increased neurofilaments. Decreased galactocerebrosidase and sphingomyelin. Immunostaining for PO protein showed clefted disruption of myelin sheaths and laminar deposits in circumferential distribution. MAG staining was similar but less definitive. Nerve conductions were normal.
MRI showed supratentorial white matter signal approximated cortex on T1, hyperintense WM on T2 (demyelination) atrophic cerebellum and thin corpus callosum.
Heterogenous Holmes-Adie syndrome has described the combination of cerebellar ataxia and hypogonadotropic hypogonadism.
Radiation retinopathy
Grimm et al. Retinopathy in survivors of primnary CNS lymphoma. Neurology 2006:67:2060-2062.
Study reports specifically five survivors of PCNSL who developed syndrome, 1.5 % of database. Patients received high dose MTX then WBRT then post-XRT chemo including procarbazine, vincristine, and carmustine. Mean latency to developing syndrome was 27 months. Note- syndrome is also reported in other diseases treated with WBRT.
Presentation was blurred vision (2/5), partial visual loss (3/5) and floaters (1/5). Fluroscein agio confirmed bilateral disease in 3. Treatments given include intraocular steroids and laser therapy. No patients had progressive ocular disease although one had radiation dementia and another had ongoing PCNSL.
Study reports specifically five survivors of PCNSL who developed syndrome, 1.5 % of database. Patients received high dose MTX then WBRT then post-XRT chemo including procarbazine, vincristine, and carmustine. Mean latency to developing syndrome was 27 months. Note- syndrome is also reported in other diseases treated with WBRT.
Presentation was blurred vision (2/5), partial visual loss (3/5) and floaters (1/5). Fluroscein agio confirmed bilateral disease in 3. Treatments given include intraocular steroids and laser therapy. No patients had progressive ocular disease although one had radiation dementia and another had ongoing PCNSL.
dopa responsive disorders
Friedman J, Hyland K, Blau N, MacCollin M. Dopa-responsive hypersomnia and mixed movement disorder due to seriapterin reductase deficiency. Neurology 2006:67:2032-2035.
Defects in monoamine synthesis are associated with phenotypes of developmental delay, diurnal variation in symptom severity, and dopa-responsive dystonia. A subset also with cognitive delay due to SPR (seriapterin reductase ) gene have dopa and serotonergic responsive hypersomnolence and movement disorder responsive.
Case report-- 27 year old woman with "CP" had delayed milestones, abnormal gait, incoordination, and hypersomnolence. Other maternal relatives had abnormal limb posturing. She had FSIQ 60, punctate cataracts, oculomotor apracis, dysarthria, bradykinesia, , PTOSIS, generalized dystonia, myoclonus, normal MRI/PET.
CSF showed decreased 5HIAA and HVA and increased 7,8 dihydropterin c/w SRD. Treatment with Sinemet and selegeline and later combined with sertraline helped.
The same patient was FIRST treated with sertraline alone (akathisia), melatonin (dystonic neck spasms), 5 hydroxytriptophan (helped but led to increased LFT's), levodopa-carbidopa (caused dyskinesias; these are rare in DRD or dopa responsive dystonias).
Inheritance is AR. Further information available at the BIODEF database (www.bh4.org)
Defects in monoamine synthesis are associated with phenotypes of developmental delay, diurnal variation in symptom severity, and dopa-responsive dystonia. A subset also with cognitive delay due to SPR (seriapterin reductase ) gene have dopa and serotonergic responsive hypersomnolence and movement disorder responsive.
Case report-- 27 year old woman with "CP" had delayed milestones, abnormal gait, incoordination, and hypersomnolence. Other maternal relatives had abnormal limb posturing. She had FSIQ 60, punctate cataracts, oculomotor apracis, dysarthria, bradykinesia, , PTOSIS, generalized dystonia, myoclonus, normal MRI/PET.
CSF showed decreased 5HIAA and HVA and increased 7,8 dihydropterin c/w SRD. Treatment with Sinemet and selegeline and later combined with sertraline helped.
The same patient was FIRST treated with sertraline alone (akathisia), melatonin (dystonic neck spasms), 5 hydroxytriptophan (helped but led to increased LFT's), levodopa-carbidopa (caused dyskinesias; these are rare in DRD or dopa responsive dystonias).
Inheritance is AR. Further information available at the BIODEF database (www.bh4.org)
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