Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 42
Filter
1.
J Neurol Neurosurg Psychiatry ; 85(8): 921-4, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24554103

ABSTRACT

BACKGROUND: A small number of patients with variant Creutzfeldt-Jakob disease (vCJD) have been treated with intraventicular pentosan polysulfate (iPPS) and extended survival has been reported in some cases. To date, there have been no reports on the findings of postmortem examination of the brain in treated patients and the reasons for the extended survival are uncertain. We report on the neuropathological findings in a case of vCJD treated with PPS. METHODS: Data on survival in vCJD is available from information held at the National CJD Research and Surveillance Unit and includes the duration of illness in 176 cases of vCJD, five of which were treated with iPPS. One of these individuals, who received iPPS for 8 years and lived for 105 months, underwent postmortem examination, including neuropathological examination of the brain. RESULTS: The mean survival in vCJD is 17 months, with 40 months the maximum survival in patients not treated with PPS. In the 5 patients treated with PPS survival was 16 months, 45 months, 84 months, 105 months and 114 months. The patient who survived 105 months underwent postmortem examination which confirmed the diagnosis of vCJD and showed severe, but typical, changes, including neuronal loss, astrocytic gliosis and extensive prion protein (PrP) deposition in the brain. The patient was also given PPS for a short period by peripheral infusion and there was limited PrP immunostaining in lymphoreticular tissues such as spleen and appendix. CONCLUSIONS: Treatment with iPPS did not reduce the overall neuropathological changes in the brain. The reduced peripheral immunostaining for PrP may reflect atrophy of these tissues in relation to chronic illness rather than a treatment effect. The reason for the long survival in patients treated with iPPS is unclear, but a treatment effect on the disease process cannot be excluded.


Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Creutzfeldt-Jakob Syndrome/drug therapy , Creutzfeldt-Jakob Syndrome/pathology , Pentosan Sulfuric Polyester/therapeutic use , Adolescent , Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Autopsy , Brain/pathology , Female , Humans , Immunohistochemistry , Injections, Intraventricular , Pentosan Sulfuric Polyester/administration & dosage , Prions/metabolism , Survival
3.
Seizure ; 11(2): 114-23, 2002 Mar.
Article in English | MEDLINE | ID: mdl-11945098

ABSTRACT

Remacemide hydrochloride is a low-affinity, non-competitive N-methyl-D-aspartic acid (NMDA) receptor channel blocker, under investigation in epilepsy. This double-blind, placebo-controlled, multicentre study assessed the safety and efficacy of remacemide hydrochloride or placebo, as adjunctive therapy, in 252 adult patients with refractory epilepsy who were already taking up to three antiepileptic drugs (including an enzyme-inducer). Patients were randomized to one of three doses of remacemide hydrochloride (300, 600 or 1200 mg /day) or placebo Q.I.D., for up to 15 weeks. An increasing percentage of responders (defined as a reduction in seizure frequency from baseline of > or =50%) was seen with increasing remacemide hydrochloride dose. At 1200 mg /day, 23% of patients were responders compared with 7% on placebo. This difference was significant (P = 0.016), as was the overall difference between treatments (P = 0.038). Adverse events: dizziness, abnormal gait, gastrointestinal disturbance, somnolence, diplopia and fatigue were mild or moderate in severity. Carbamazepine and phenytoin plasma concentrations were well controlled and maintained within target ranges, with no evidence of improved seizure control due to increases in the concentrations of these drugs. A dose-dependent, significant, increase in responders following adjunctive remacemide hydrochloride compared with placebo was observed. Remacemide hydrochloride was well tolerated.


Subject(s)
Acetamides/administration & dosage , Anticonvulsants/administration & dosage , Epilepsy/drug therapy , Acetamides/adverse effects , Acetamides/blood , Adolescent , Adult , Aged , Anticonvulsants/adverse effects , Anticonvulsants/blood , Carbamazepine/administration & dosage , Chi-Square Distribution , Dose-Response Relationship, Drug , Double-Blind Method , Drug Administration Schedule , Drug Therapy, Combination , Epilepsy/blood , Female , Humans , Male , Middle Aged , Outcome Assessment, Health Care , Phenytoin/administration & dosage , Statistics, Nonparametric
6.
Mov Disord ; 15(2): 318-20, 2000 Mar.
Article in English | MEDLINE | ID: mdl-10752584

ABSTRACT

Gilles de la Tourette syndrome is characterized by vocal and motor tics starting in childhood. Vocal tics may be either noises or words, and the vocal language tics may consist of obscenities (coprolalia) and repetitions of speech that has been heard (echolalia). We describe a prelingually deaf man who has the full array of tics seen in Gilles de la Tourette syndrome, but in whom vocal language tics are replaced by equivalent sign language tics. This is, to our knowledge, the first report of sign language tics in a person with prelingual deafness. The implications of this phenomenon for the separation between language and ideas in tics and the equivalence of sign language to spoken language is discussed.


Subject(s)
Deafness/diagnosis , Sign Language , Tourette Syndrome/diagnosis , Adult , Autistic Disorder/diagnosis , Follow-Up Studies , Humans , Male , Semantics , Verbal Learning
11.
J Neurol Neurosurg Psychiatry ; 61(4): 369-75, 1996 Oct.
Article in English | MEDLINE | ID: mdl-8890775

ABSTRACT

Five patients with clinically definite multiple sclerosis are reported who presented with acute relapses associated with hypothermia. Repeated episodes of hypothermia were seen in four. Thrombocytopenia was associated with the hypothermia in four patients. Further investigation disclosed a tendency to chronic hypothermia and suggested an altered thermoregulatory set point in one patient, when MRI, endocrine, and autonomic studies failed to localise a lesion in the hypothalamus, but subsequent necropsy showed hypothalamic lesions. In such patients a predisposition to altered thermoregulation may occur due to direct involvement of the hypothalamus or from combined lesions affecting hypothalamic outflow to the brainstem and spinal cord.


Subject(s)
Hypothermia/complications , Multiple Sclerosis/complications , Adult , Brain Stem/physiopathology , Female , Humans , Hypothalamus/physiopathology , Hypothermia/diagnosis , Magnetic Resonance Imaging , Male , Middle Aged , Multiple Sclerosis/physiopathology , Recurrence , Spinal Cord/physiopathology , Thrombocytopenia/complications
13.
J Clin Pharm Ther ; 19(4): 219-22, 1994 Aug.
Article in English | MEDLINE | ID: mdl-7989400

ABSTRACT

Steroids are widely used in treating the relapse of multiple sclerosis. There is no place for long term steroid therapy of this disease. Current practice is a short course of high dose methylprednisolone which can be repeated after an interval, has proven safety and objectively accelerates recovery.


Subject(s)
Methylprednisolone/therapeutic use , Multiple Sclerosis/drug therapy , Cost-Benefit Analysis , Drug Administration Schedule , Drug Delivery Systems , Drug Evaluation , Humans , Methylprednisolone/administration & dosage , Methylprednisolone/pharmacology , Recurrence
14.
J Neurol Neurosurg Psychiatry ; 56(11): 1219-20, 1993 Nov.
Article in English | MEDLINE | ID: mdl-8229035

ABSTRACT

A randomised double-blind placebo-controlled trial of intravenous methylprednisolone versus oral methylprednisolone at equivalent high dose was carried out on 35 patients with an acute relapse of multiple sclerosis (MS). After baseline evaluation each was randomly allocated to oral treatment and intravenous placebo or intravenous treatment and oral placebo, receiving 500 mg of methylprednisolone for five consecutive days and with reassessment at days five and twenty-eight. There was no significant difference in response when disability or functional scores were compared in the two groups. Adverse effects were minor and equally distributed. In this study oral treatment with methylprednisolone was as effective as intravenous treatment in acute relapse of MS.


Subject(s)
Methylprednisolone/administration & dosage , Multiple Sclerosis/drug therapy , Administration, Oral , Adult , Double-Blind Method , Female , Humans , Infusions, Intravenous , Male , Middle Aged , Placebos
16.
J Neurol Neurosurg Psychiatry ; 54(3): 200-3, 1991 Mar.
Article in English | MEDLINE | ID: mdl-2030345

ABSTRACT

A community based survey was undertaken to assess the work record of patients with epilepsy in an area of high unemployment. One hundred and thirty seven epileptic patients were identified from a population of 23,837 persons of employable age registered with three urban group practices in North East England. The unemployment rate for economically active patients with epilepsy was 46% compared with 19% for an age and sex matched control population (p less than 0.01). Fifty nine per cent of patients with active epilepsy were unemployed. In those with an associated neurological or psychiatric handicap and those who were unskilled manual workers the unemployment rates were 79% and 77%, respectively. Patients with epilepsy were less likely to leave school with qualifications or undergo subsequent training or apprenticeships. They were more likely to be unskilled manual workers, single and living in rented accommodation. In an area of high unemployment patients with epilepsy have disproportionately greater difficulty finding work. High unemployment rates among patients with epilepsy are only one aspect of a spectrum of social and economic disadvantage.


Subject(s)
Epilepsy/rehabilitation , Rehabilitation, Vocational/statistics & numerical data , Unemployment/statistics & numerical data , Urban Population/statistics & numerical data , Adolescent , Adult , Cross-Sectional Studies , Disability Evaluation , England/epidemiology , Epilepsy/epidemiology , Female , Humans , Incidence , Male , Middle Aged , Neurologic Examination
17.
Neurology ; 41(2 ( Pt 1)): 228-33, 1991 Feb.
Article in English | MEDLINE | ID: mdl-1992366

ABSTRACT

We describe 5 patients with a relapsing encephalopathy in association with Hashimoto's disease and high titers of anti-thyroid antibodies. The presentation is usually with a subacute onset of confusion, alteration in conscious level, and focal or generalized seizures. The relapsing course, association with myoclonus or tremulousness, and episodes of stroke-like deterioration are characteristic features. The long-term prognosis is favorable with steroid therapy, though additional immunosuppressive therapy may be required. Neurologic investigation typically shows a diffusely abnormal EEG, high CSF protein level without pleocytosis, and normal brain CT and cerebral angiogram. Isotope brain scan may show patchy abnormal uptake. Hashimoto's encephalopathy should be recognized as a definite neurologic entity and added to the list of CNS complications of thyroid disease.


Subject(s)
Autoantibodies/analysis , Brain Diseases/etiology , Steroids/therapeutic use , Thyroid Gland/immunology , Thyroiditis, Autoimmune/complications , Adolescent , Brain Diseases/diagnostic imaging , Brain Diseases/physiopathology , Electroencephalography , Female , Humans , Middle Aged , Recurrence , Thyroiditis, Autoimmune/drug therapy , Thyroiditis, Autoimmune/immunology , Tomography, X-Ray Computed
18.
BMJ ; 301(6749): 395-6, 1990 Sep 01.
Article in English | MEDLINE | ID: mdl-2282391
SELECTION OF CITATIONS
SEARCH DETAIL
...