Clinical speech impairment in Parkinson's disease, progressive supranuclear palsy, and multiple system atrophy.

Context: Speech abnormalities are common to the three Parkinsonian syndromes, namely Parkinson's disease (PD), progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), the nature and severity of which is of clinical interest and diagnostic value. Aim: To evaluate the clinical pattern of speech impairment in patients with PD, PSP and MSA and to identify significant differences on quantitative speech parameters when compared to controls. Design and Setting: Cross-sectional study conducted in a tertiary medical teaching institute. Materials and Methods: Twenty-two patients with PD, 18 patients with PSP and 20 patients with MSA and 10 age-matched healthy controls were recruited over a period of 1.5 years. The patients were clinically evaluated for the presence and characteristics of dysarthria. This was followed by quantitative assessment of three parameters: maximum phonation time (MPT), semantic fluency and reading speed. The outcome measures were compared between the patient groups and with controls. Results: Patients with PD had hypophonic monotonous speech with occasional rushes of speech while patients with MSA and PSP had mixed dysarthria with ataxic and spastic elements respectively. All quantitative parameters were affected when compared to controls ( P values < 0.001, 0.012 and 0.008 respectively). Maximum phonation time was significantly less in PSP when compared to MSA and PD ( P =0.015). Reading speed also showed a similar trend which was not statistically significant. Semantic fluency was comparable in all three groups. Conclusion: Dysarthria in PD, PSP and MSA have many overlapping but certain distinctive features as well which could serve as a diagnostic clue. Patients with PSP had profound speech impairment probably indicative of the more severe frontostriatial pathology.

Hyperacute thrombolysis with IV rtPA of acute ischemic stroke: efficacy and safety profile of 54 patients at a tertiary referral center in a developing country.

BACKGROUND: Given the constraints of resources, thrombolysis for acute ischemic stroke (AIS) is under evaluation in developing countries. Prothrombin time (PT), platelet count and activated partial thromboplastin time (aPTT) may not be feasible within the time window. AIM: To evaluate the safety and efficacy of thrombolysis in selected patients without the coagulation profile. DESIGN: Open, nonrandomized, observational study. MATERIALS AND METHODS: Fifty-four stroke patients were classified using TOAST criteria (large artery atherosclerotic = 13; cardioembolic = 12; small vessel occlusion = 22; other determined etiology =three; undetermined etiology = four). The mean time to reach emergency was 2.4h (1.15-3.4), the mean door to CT, 24 min (10-47) and the door to recombinant tissue plasminogen activator (r-tPA) injection, 26.8 min (25-67). The NIHSS scores ranged from 11 to 22 (mean = 15.5 +/- 2.7). Patients with history of liver or renal disease or those on anticoagulants were excluded. The PT, aPTT and platelet count were not done. Recombinant tissue plasminogen activator was administered at a dosage of 0.9 mg/Kg. RESULTS: Thirty-five patients (65%) significantly improved on NIHSS at 48 h (> or =4 points) (mean change = 10; range= 4-17). At one month, 43 (79%) improved on Barthel Index (mean change = 45%). One each developed small frontal lobe hemorrhage and recurrent stroke; one died of aspiration; and eight showed no improvement. CONCLUSIONS: Hyperacute thrombolysis was found useful and safe in selected patients with AIS even without the coagulation studies.

Role of CSF serology in follow-up of subacute sclerosing panencephalitis patients on treatment.

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system with poor prognosis and high mortality. No effective treatment has a proven role; oral isoprinosine and intrathecal administration of alpha-interferon may prolong survival. We report an unusual case of adult onset SSPE patient on treatment with significant clinical improvement, even in the absence of conversion to seronegativity in either CSF or serum, on follow-up serological examination.

Erythrocyte antioxidant enzymes in Parkinson's disease.

BACKGROUND & OBJECTIVES: Oxidative stress is incriminated to play a central role in the pathogenesis of Parkinson's disease (PD). Oxidative stress, to which neurons are highly susceptible, is also known to induce oxidative changes in human red blood cells (RBCs), in vivo and in vitro. Earlier studies on oxidative stress in RBCs in patients with PD have yielded controversial results claiming unaltered activity to reduced activity. Using RBC as a model, we have undertaken this study to ascertain the possibility of oxidative damage to the RBCs in PD by measuring the cytosolic antioxidant enzymes viz., superoxide dismutase (SOD), catalase (CAT) glutathione peroxidase (G-Px) and glucose-6-phosphate dehydrogenase (G6PD). METHODS: Activities of antioxidant enzymes were measured in erythrocytes of 115 PD patients and 37 normal age-matched healthy persons as controls. Enzymes activities were correlated with age of patients, age of onset of disease, duration of disease, United Parkinson's Disease Rating Scale (UPDRS) and Hoehn and Yahr stage. RESULTS: The SOD, CAT, G-Px and G6PD activities were significantly lower in patients with PD compared to the control. A significant (P<0.05) negative correlation of enzyme activities with Hoehn and Yahr stage of the disease and also with UPDRS score was found. INTERPRETATION & CONCLUSION: Results of the present study showed involvement of oxidative stress as one of the risk factors, which can initiate and/or promote neurodegeneration in PD and was correlated to the severity of the disease.

Myasthenic crisis: a retrospective study.

BACKGROUND AND OBJECTIVE: Myasthenic crisis (MC) is one of the important and common complications in the natural history of myasthenia gravis (MG). MC can be precipitated by multiple factors including deficiency or excess of the acetylcholinesterase inhibitors (AChEI). Any episode of MC is an emergency requiring aggressive therapy. We studied the demographic, clinical and treatment-related characteristics of patients who developed MC. MATERIALS AND METHODS: A retrospective study was conducted in patients with MC admitted during a 31-month period from February 1999 to August 2001, at a tertiary care center in India. RESULTS: Eleven patients (9.69% of the total 114 patients with MG) were admitted with 12 episodes of MC. Mean age at presentation was 39.83 + 13.18 years with male predominance. Seven patients had undergone thymectomy previously; of which 2 had postoperative MC. Six patients had thymoma. Steroid or cholinesterase inhibitor withdrawal and infections were the commonest precipitating factors for MC. Patients required ventilatory support for median 14 days. They responded to low volume of plasma exchange (PE) (mean 854 ml / day with mean 6.5 cycles per patient). CONCLUSIONS: This report highlights that the subset of Indian patients with MG who are at risk to develop MC belong to the 3rd and 4th decade, have bulbar symptoms at presentation and are associated with thymoma. Patients with MC should have judicious drug adjustments under supervision and should be treated aggressively during impending MC.

Leigh's syndrome.

A 15-month-old female child presented with sudden onset cough and hyperventilation along with evidence of metabolic acidosis. She had past history of recurrent vomiting, episodes of abnormal posturing, difficulty in deglutition and regression of milestones since 12 months of age. CT scan of the brain revealed hypodense lesions in bilateral basal ganglia and on MRI there were T2 hyperintensities in bilateral lentiform nuclei, caudate nuclei, thalamus, red nuclei and dentate nuclei. Biochemical examination revealed persistently elevated serum lactate levels with high lactate/pyruvate ratio. Resuscitative measures were of no avail and the child succumbed to the illness on the second day of admission. Neuropathological examination at autopsy demonstrated marked spongiosis, focal necrosis, endothelial proliferation, reactive astrogliosis and extensive demyelination involving bilateral basal ganglia, midbrain and spinal cord which were typical of Leigh's sub acute necrotizing encephalomyelopathy.

Increased depressant effect of phenytoin sodium as compared to carbamazepine on cortical excitability: a transcranial magnetic evaluation.

To evaluate the effect of monotherapy (phenytoin sodium (DPH) and carbamazepine (CBZ) on the threshold intensity (TI), cortical latency (CL), central conduction time (CCT), using transcranial magnetic stimulation (TMS). A single pulse transcranial magnetic stimulation was used for recording the motor-evoked potentials (MEP) from the thenar muscles of both hands, in 36 patients with well-controlled epilepsy on monotherapy, with normal EEG and imaging studies. The TI, CL, CCT and the MEP amplitude were recorded and compared with 20 healthy controls. The threshold intensity was significantly higher in patients on DPH, (P< 0.05) with a significant decrease in the MEP amplitude when compared with controls (P< 0.05). Anticonvulsants alter the excitability of human motor pathways in epileptic subjects. This effect differs among the drugs used; DPH had a greater depressant effect on the excitability than CBZ in the present study.

Neuronal stem cells.

Stem cells are self regenerating multipotential cells, found in the human brain which have the potential to differentiate into neurons, astrocytes and oligodendrocytes, and to self renew sufficiently to provide adequate number of cells in the brain. Neural stem cell grafts have been studied in a variety of animal models for various diseases like metabolic disorders, muscular dystrophies, neurodegenerative disorders, spinal cord repair, brain tumors and demyelinating disease. Stem cells may be derived from autologus, allogeneic or xenogenic sources. Histocompatibility is prerequisite for transplantation of allogeneic stem cells. Fetal tissue is the best current tissue source for human neural stem cells, however ethical issues are a major concern. Thus the prospect that stem cells could potentially be used to promote neurogenesis following injury and disease may seem attractive, yet the inherent problems associated with isolation and rejection in case of stem cells from another source, the potential to form tumors and ethical issues are the major challenges.

Carpal tunnel syndrome after 22 years of Colle's fracture.

Delayed carpal tunnel syndrome is rare. We describe the electrophysiological findings in a patient with Colle's fracture, who developed carpal tunnel syndrome 22 years after a wrist injury.

Increased cortical excitability with longer duration of Parkinson's disease as evaluated by transcranial magnetic stimulation.

Transcranial Magnetic Stimulation (TMS) was used to evaluate the cortical excitability and central motor pathways in Parkinson's disease (PD) and correlate with severity and duration of disease. 19 cases of PD and 13 controls were enrolled. The threshold intensity (TI), cortical latency (CL), central conduction time (CCT), motor evoked potential amplitude (MEP) obtained with TMS were correlated with Hoehn and Yahr and duration of disease. The threshold intensity (TI) was significantly lower in patients of PD than controls. The TI in patients with PD was 53.16-/+8.4% patients and 67.1-/+21.6% in controls (p<0.05). This strongly correlated with duration of disease, TI being lower in patients with disease duration more than 5 years. There was no difference in the other TMS parameters - CL, CCT, MEP between patients and controls. Our study revealed increased excitability in PD which was related to longer duration of disease.

Cerebral venous thrombosis in ulcerative colitis.

Cerebral venous thrombosis is a rare complication of ulcerative colitis. We report a case of 29 year old male who developed superior sagittal, left lateral and sigmoid sinus thrombosis secondary to ulcerative colitis. He was successfully treated with low molecular weight heparin and steroids.

Dystonia as presenting manifestation of ataxia telangiectasia : a case report.

Ataxia telangiectasia is a genetically inherited multisystem disorder with predominant feature being telangiectasia and cerebellar ataxia. In this report, a family of three siblings suffering from ataxia telangiectasia is described. The proband presented with dystonia and dystonic myoclonus, both of which are rare presenting features of ataxia telangiectasia.

Serum cobalamin levels in dementias.

The present study attempts to find a correlation between low serum B-12 levels and dementias. A total of 178 patients of dementia were seen at the cognitive disorder clinic from 1996 to 1998. Serum B-12 levels were studied in 100 patients. 15 patients (39.5%) of Alzheimer's dementia had reduced B-12 levels, while only 5 patients (13.9%) with vascular dementias and 3 patients (11.5%) with other types of dementias had reduced levels. The incidence of low serum B-12 was statistically significant in the Alzheimer's group when compared with the other groups, individually as well as combined together (p<0.05). The aetiopathogenesis and significance of these findings is discussed and the literature is reviewed.

Physician assisted suicide--the perspective of a neurologist.

Modern medicine has resulted in a better control and treatment of many diseases. This has provided a better life for patients with treatable disorders, but at the same times has prolonged the suffering of the patients with diseases that are not treatable. This is of particular importance for the patients with neurological diseases, as some of these conditions have a relentlessly progressive course and cause significant distress to the patient. Recent times have seen an expansion of the scope of the various "rights of the patient" and according to some, this includes the "right to die". As a result of this concept of regarding the autonomy of the patient, the phenomenon of physician assisted suicide has emerged and is a topic of intense debate amongst the physicians, social workers and politicians all over the world. The present article puts forth the current concepts and brings out the exact definitions of the terms like physician-assisted suicide, voluntary active euthanasia and voluntary passive euthanasia. It presents the statements made by the various societies and organisations regarding the PAS. It also brings forth the point of view of the authors about this aspect of medicine. The authors share the view of the various scientific organisations, and opine that though the idea of physician assisted suicide may be morally justifiable in certain circumstances, it should not be made legal.

Interictal brain 99m Tc-HMPAO SPECT study in cases of epilepsy with single ring enhancing CT lesion.

BACKGROUND: Contrast enhancing single ring or disc lesion (CESRL), a frequent finding in the CT scan of Indian patients with seizures, has a diverse etiology. Underlying cause in many of these cases remains conjectural. Some of these ring lesions show spontaneous resolution without any specific treatment and in others their temporal profile remains unpredictable. MATERIAL AND METHODS: In a prospective study we studied 17 cases of epilepsy, with the CT scan finding of single contrast enhancing ring lesion. Detailed clinical evaluation and interictal EEG was performed. CT scan and SPECT study was done initially and were then repeated. RESULTS: On the second CT, one lesion disappeared and 2 reduced in size, three showed no change in size. Third CT showed complete disappearance in one case and no change in the second case. Initial SPECT study was abnormal in all 17 cases, showing areas of hypoperfusion corresponding to the anatomical location of ring lesion. On follow up, patients with decreased ring size on CT showed decrease in area of perfusion deficit on the SPECT, but cases in which the lesion disappeared on the CT, the SPECT perfusion abnormality continued to persist, though to a lesser extent. CONCLUSION: Persistence of perfusion defects suggest the presence of altered underlying physiology. Hence, early withdrawal of antiepileptic drugs after disappearance of ring lesion on CT may lead to seizure recurrence. SPECT studies repeated after 3 months may help to prognosticate cases with CESRL and also help in deciding the optimum duration of antiepileptic therapy in individual cases.