Hashimoto's encephalopathy: clinical, SPECT and neurophysiological data.
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Adult↗
Biomedical subjects
U K Misra
Publications and source records attributed to U K Misra.
Bladder rupture: a rare complication of tonic-clonic seizures.
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Humans↗
Thallium poisoning: emphasis on early diagnosis and response to haemodialysis.
Thallium poisoning is known for its diverse manifestations and these can delay the diagnosis if a clear history of poisoning is not forthcoming. A 42 year old man presented on the third day of illness with flaccid quadriparesis and paresthesia, which were confused with Guillain-Barré syndrome. Because of associated loose motions, skin lesions, and liver and kidney dysfunction arsenic poisoning was considered. In the second week he developed ophthalmoplegia, nystagmus, and neck tremor and later developed alopecia, and thallium poisoning was suspected. His serum thallium level on the 18th day of illness was 40 980 micro g/ml. He was subjected to haemodialysis, potassium supplementation, laxatives, and B complex supplementation. He showed significant improvement after haemodialysis and at three months he was able to walk with support. At six months of follow up he was independent for activities of daily living. Severe paresthesia, ophthalmoplegia, cerebellar and extrapyramidal signs, and alopecia are highly suggestive of thallium poisoning. Haemodialysis may be effective even in the third week of poisoning.
Adult↗
Evoked potential changes in ischaemic myelopathy.
Somatosensory evoked potentials are employed in the intraoperative monitoring but there is paucity of information about the motor evoked potentials (MEP) in spinal cord ischaemia. Two patients aged 9 and 4 years developed paraplegia following surgery for coarctation of aorta. The aortic cross clamping time in these patients was 100 min and 30 min. respectively. The patient with longer clamping time had flaccid paraplegia and lower limb MEPs were not recordable; whereas the patient with shorter clamping time had spastic paraparesis and prolonged CMCT to right lower limb. The latter patient improved significantly. Tibial SEPs were normal in both the patients. Our results suggest that MEP may have a greater role than SEP in documenting and prognosticating ischaemic myelopathy.
Aortic Coarctation↗
Clinical, radiological and neurophysiological spectrum of JEV encephalitis and other non-specific encephalitis during post-monsoon period in India.
AIMS: To study the spectrum of encephalitis during the post-monsoon period in a tertiary care centre of India. METHODS: Clinical, neurophysiological and radiological features of patients with encephalitis are reported in this communication. The patients were subjected to clinical examination, CT or MRI scan, EEG, motor and somatosensory evoked potentials in both upper and lower limbs bilaterally and concentric needle electromyography. The laboratory studies for Japanese encephalitis (JE) comprised virus isolated, IgM capture ELISA, mercaptoethanol test and hemagglutination inhibition titre in paired sera against JE virus. Patients were classified into JEV encephalitis and non-specific encephalitis. On the basis of radiological features, they were classified into group I (thalamic or basal ganglia involvement), group II (brainstem involvement only) and group III (normal MRI). The outcome was defined into poor (bedridden), partial (dependent for daily activities) and complete (independent) recovery at the end of 3 months. RESULTS: Out of 26 patients (Age 7-70 years, mean 24.8 years), laboratory evidences of JEV infection was present in 14 patients and one patient had herpes simplex encephalitis. The patients with JEV encephalitis had more severe illness as evidenced by lower GCS score, higher frequency of anterior horn cell involvement, movement disorders and more extensive MRI changes. The EEG and MEP changes were also more frequently abnormal in the JEV group. On radiology, 15 patients had thalamic or basal ganglia involvement (group I), 3 isolated midbrain involvement (group II) and 8 had normal MRI (group III). Laboratory evidence consistent with JE were present in 11 out of 12 patients in group I and 3 out of 8 in group III, however, there was no laboratory evidence of JE virus infection in patients with isolated brainstem involvement. There was overlap in the neurologic and systemic manifestations in all the 3 radiological groups as well as in the groups with and without laboratory evidences of JEV infection. CONCLUSION: The observed overlap in neurological and systemic involvement in different subgroups of encephalitis may be due to JE or JE-like viral infection. The possibility of strain variation, change in virulence of organism or immunity of host needs further studies.
Adolescent↗
Current status of osmotherapy in intracerebral hemorrhage.
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Cerebral Hemorrhage↗
Sporadic variety of pallido-pyramidal syndrome.
A rare case of a 40-year-old lady with a sporadic variety of the pallido-pyramidal syndrome (PPS) is reported. She had marked parkinsonian features on the left side. Her single photon emission computed tomography showed left frontoparietal and basal ganglia hypoperfusion. CT scan and central motor conduction time were normal. She responded partially to a combination of trihexyphenydil and L dopa/C dopa therapy. In view of the diversity in the genetic, clinical and laboratory features, it is possible that PPS may be a heterogeneous condition.
Adult↗
Phenytoin and carbamazepine cross reactivity: report of a case and review of literature.
Cross reactivity between phenytoin, carbamazepine, and oxcarbazepine is reported. An 8 year old boy with partial seizures developed maculopapular rashes with itching on day 15 of carbamazepine therapy. After stopping carbamazepine, phenytoin 100 mg daily was prescribed two days later. On the 12th day of phenytoin therapy he developed cervical and axillary lymphadenopathy with fever. Lymph nodes revealed reactive hyperplasia. Oxcarbazepine 75 mg twice daily also resulted in oral and mucosa ulceration. The seizures were controlled without any side effects with sodium valproate 200 mg three times a day and gabapentin 300 mg twice a day. Due to the cross reactivity of aromatic anticonvulsants (phenytoin, carbamazepine, and oxcarbazepine), valproate or newer anticonvulsants should be used if a patient has sensitivity to these drugs.
Anticonvulsants↗
Clinicoradiological observation in three patients with suicidal hanging.
STUDY DESIGN: A hospital-based clinical, radiological and neurophysiological study. OBJECTIVES: Hanging is a common mode of suicide in India, but there is paucity of MRI and neurophysiological findings in the context of clinical and neurological changes. We report the clinical, neurophysiological and MRI studies of three females who attempted suicide by hanging. METHODS: All three patients who attempted hanging underwent detailed clinical, electroencephalographic, motor, somatosensory and brainstem-evoked potential and magnetic resonance imaging studies. Patients were clinically followed up for 6 months. RESULTS: Their ages were 21, 26 and 35 years. They developed altered sensorium, decorticate posturing and had a wide variety of movement disorders. Patient no. 1 had the most severe illness and recovered by the seventh month while the others recovered earlier. MRI revealed hyperintense signal changes in the globus pallidus, caudate nucleus and thalamus in all patients and in the midbrain in one patient. Electroencephalography showed nonspecific slowing. Somatosensory, motor and brainstem-evoked potential studies were normal. CONCLUSION: Hanging leads to hypoxic brain damage resulting in signal changes mainly in the basal ganglia and thalamus and may be associated with transient movement disorders.
Adult↗
Myelopathy and amnesia following accidental electrical injury.
OBJECTIVE: Documentation of MRI and neurophysiological changes following accidental electrical injury. SETTING: Tertiary care referral teaching hospital at Lucknow, India. RESULTS: A 30-year-old lady developed amnesia and spastic paraparesis with loss of pin prick sensation below the second thoracic spinal segment following electrocution. Her spinal MRI was normal and cranial MRI revealed T2 hyperintensity in the right putamen. Peroneal, sural and electromyography were normal. Tibial central sensory conduction time was normal but central motor conduction time to lower limbs and right upper limb was prolonged. CONCLUSION: Neurophysiological study and MRI may help in understanding the pathophysiological basis of neurological sequelae following electrical injury.
Adult↗
Bladder dysfunction in acute transverse myelitis: magnetic resonance imaging and neurophysiological and urodynamic correlations.
AIMS: To evaluate micturition abnormalities in acute transverse myelitis and correlate these with evoked potentials, magnetic resonance imaging (MRI), and urodynamic findings. SETTING: Tertiary care teaching hospital. PATIENTS: 18 patients with acute transverse myelitis, aged 4-50 years; 15 had paraparesis and three quadriparesis. METHODS: Patients with acute transverse myelitis had a neurological evaluation and tibial somatosensory and motor evoked potential studies in the lower limbs. Spinal MRI was carried out using a 1.5 T scanner. Urodynamic studies were done using Dantec UD 5500 equipment. Neurological outcome was determined on the basis of Barthel index score at six months as poor, partial, or complete. In some patients, urodynamic studies were repeated at six and 12 months. RESULTS: Spinal MRI in 14 of the 18 patients revealed T2 hyperintense signal changes extending for at least three spinal segments in 13; one patient had normal MRI. In the acute stage, 17 patients had a history of urinary retention and one had urge incontinence. On follow up at six months two patients regained normal voiding, retention persisted in six, and storage symptoms developed in 10, of whom five also had emptying difficulties. Urodynamic studies showed an areflexic or hypocontractile bladder in 10, detrusor hyperreflexia with poor compliance in two, and detrusor sphincter dyssynergia in three. Early abnormal urodynamic findings commonly persisted at the six and 12 months examinations. Persistent abnormalities included detrusor hyperreflexia, dyssynergia, and areflexic bladder. The urodynamic abnormalities correlated with muscle tone and reflex changes but not with sensory or motor evoked potentials, muscle power, MRI signal changes, sensory level, or six months outcome. CONCLUSIONS: Bladder dysfunction is common in acute transverse myelitis and may be the only sequel. Urodynamic study is helpful in evaluating the bladder dysfunction and also in its management.
Adolescent↗
Prognosis of Japanese encephalitis patients with dystonia compared to those with parkinsonian features only.
OBJECTIVES: A number of movement disorders have been reported in Japanese encephalitis (JE). The prognostic significance of these movement disorders, however, has not been evaluated. The present study reports the prognostic significance of parkinsonian features and dystonia in JE. PATIENTS AND METHODS: During 1992 and 1998, 50 JE patients were managed; 35 of them developed movement disorders (the study group). The diagnosis of JE was based on clinical, radiological, and serological criteria. Parkinsonian features were rated by the unified Parkinson's disease rating scale and dystonia by the dystonia rating scale. The patients with parkinsonian features only were classified into group I and those with additional dystonia or dyskinesia into group II. The outcome was defined at the end of three months into poor, partial, and complete recovery depending on how the patients coped with daily living activities. RESULTS: The patients' ages ranged from 2 to 64 years and 11 were females. The admission mean Glasgow coma scale score was 6.9 (range 4-13). The movement disorders were noted after 1-4 weeks of ictus. There were 16 patients in group I and 19 in group II. The parkinsonian features were more pronounced in group II than in group I. At three months of follow up, fewer patients had parkinsonian features in group I than group II. Hypophonia, however, persisted in 12 patients in group I and 16 in group II until the three month follow up. In group II, the mean dystonia score was 3.2 which regressed to 1.8 at three months. Tremor was present in five patients in groups I and eight in group II. Cranial computed tomography was abnormal in six and magnetic resonance imaging abnormal in 15 patients in group I and in nine and 12 patients respectively in group II. The thalamus was most frequently involved (11 patients in each group), basal ganglia (four in group I and six in group II), and midbrain (six in group I and one in group II). Group II patients had poorer recovery compared with group I. In group I, at the end of three months functional recovery was complete in 10, partial in two, and poor in three patients. In group II, four patients had complete, seven partial, and eight poor recovery. CONCLUSION: JE results in a transient form of parkinsonian syndrome, which is associated with a lower frequency of tremor and prominent hypophonia. The presence of dystonia suggests more severe illness and poorer prognosis.
Adolescent↗
Idiopathic hypertrophic cranial pachymeningitis: easy to suspect, difficult to prove.
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Brain Diseases↗
Neurophysiological changes in Japanese encephalitis.
Japanese encephalitis (JE) is an encephalomyelitis involving cortex, subcortex, brainstem and spinal cord. There is paucity of studies on the neurophysiological evaluation in JE. This study aims at comprehensive evaluation of EEG, sensory and motor evoked potentials, nerve conduction and electromyography; and correlate these with clinical findings. Sixty five patients with JE diagnosed on the basis of clinical, radiological and virological criteria were subjected to a detailed clinical evaluation during the acute stage of illness. Cranial CT scan or MRI was carried out in all the patients. All the patients underwent 10 or 18 channel EEG, motor and sensory evoked potentials to both upper and lower limbs bilaterally as well as peroneal and sural nerve conductions and concentric needle EMG. Outcome, was defined at the end of 3 months into poor, partial and complete recovery. The patient's age ranged between 2-65 years. There were 40 males and 25 female patients. Fifteen patients were less than 12 years of age. History of seizure was present in 31 patients. Quadriplegia was seen in 39 and hemiplegia in 8 patients. Muscle wasting was present in 16 patients and tendon reflexes were reduced in 12 and of mixed pattern in 14 patients. Cranial MRI revealed thalamic lesion in 38, basal ganglia in 21, substantia nigra in 30, pons in 5, cerebellum in 3 and cerebral cortex in 7 patients out of 57 patients. EEG revealed nonspecific theta to delta slowing in 45, alpha pattern coma in 5 and epileptiform discharges in 8 patients. EMG revealed fibrillations in 23 patients. Motor evoked potentials were abnormal in 34 out of 46 patients and revealed patchy and focal abnormalities comprising of unrecordable, prolonged and normal pattern. Somatosensory evoked potentials were abnormal in 8 patients only. At 3 month, 26 patients had complete, 13 partial and 15 had poor outcome. Eight patients died in acute stage and 3 were lost to followup. MEP correlated with weakness and 3 month outcome whereas EEG, SEP and EMG did not have any correlation. MEP changes were more frequent in JE and had prognostic significance.
Adolescent↗
Landau Kleffner syndrome: electroclinical and etiopathogenic heterogeneity.
Landau - Kleffner syndrome is a rare, functional, age-related epilepsy with aphasia and epileptiform discharges on EEG. The heterogenity of clinical presentations, course, long-term outcome and response to treatment suggests multiple underlying etiologies. Normal children abruptly develop deterioration of language functions along with spike and wave discharges on EEG. Clinical seizures may or may not be present. The aphasia responds poorly to most drugs. Valproic acid and benzodiazepines are most effective. Steroids and intravenous immunoglobulins have shown a variable response. Long-term outcome of aphasia is variable, many patients persist with residual impairment. Important questions regarding etiopathogenesis are unanswered.
Anticonvulsants↗
Receptor-associated protein binding blocks ubiquitinylation of the low density lipoprotein receptor-related protein.
The low density lipoprotein receptor-related protein (LRP) consists of two subunits, M(r) approximately 515,000 and 85,000. LRP is a receptor for activated alpha2-macrogobulin (alpha2M*), Pseudomonas exotoxin A, and many other proteins. We now report that ubiquitinylation of the LRP heavy chain occurred when either Pseudomonas exotoxin A or alpha2M* bound to LRP on macrophages. Ubiquitinylation was dose-dependent and maximal about 30 min after ligation of the receptor. Addition of the proteosome inhibitor MG-132 sustained the level of ubiquitin-LRP for longer time intervals in macrophages treated with either alpha2M* or Pseudomonas exotoxin A. By contrast, when receptor associated protein (RAP) bound to LRP, ubiquitinylation did not occur. While RAP is not found in the extracellular environment it binds to LRP and is believed to function as an intracellular chaperone. The presence of RAP within the cell may, therefore, contribute to the recycling of intact LRP which has ligated and internalized its ligands.
ADP Ribose Transferases↗
Seizures in Japanese encephalitis.
OBJECTIVE: Although Japanese encephalitis (JE) has been reported to be associated with seizures, there is no report on its frequency, pattern and severity and their correlation with electroencephalography (EEG) and radiological findings. In this communication, the analysis of seizures in 65 JE patients seen during 1991-1999 is presented. METHODS: The diagnosis of JE was based on clinical, radiological and serological criteria. The patients were subjected to a detailed clinical evaluation. Frequency and pattern of seizures and other neurological findings were recorded. Cranial CT scan or MRI was carried out in all the patients. Electroencephalograph was recorded using the 10-20 system of electrode placement. Patients' outcome was defined, on the basis of clinical status for 3 months, into poor, partial and complete recovery. The relationship between seizures and other clinical, radiological and EEG parameters was evaluated by the Z test of proportion using the two-tail approach. RESULTS: The patient's age ranged between 2 and 57 years, 24 were females and 19 were below 13 years of age. Thirty patients had seizures within the first week of encephalitis. The seizures were generalised tonic-clonic in 17 patients and partial motor with secondary generalization in 13 patients. Eleven patients had a single seizure, 8 had two seizures and 11 had multiple seizures. Two patients were presented with status epilepticus. Interictal EEG in the patients with seizures revealed theta to delta slowing in all patients and epileptiform discharges in four patients only. MRI revealed thalamic lesions bilaterally in 24, cortical in 7, basal ganglia in 8 and brainstem in 3 patients out of 26 patients with seizure. In the seizure group, three patients died, nine had poor, eight had partial and nine had complete recovery. Upon comparison of the JE patients with and without seizures, Glasgow coma scale (GCS) score, focal weakness, EEG slowing and cortical and thalamic lesion on CT or MRI were significantly related to the occurrence of the seizures; however, it was not associated with poor outcome. CONCLUSIONS: JE is associated with seizures in 46% of the patients in the acute stage of encephalitis which is easily controlled by monotherapy. Patients with severe encephalitis were associated with higher frequency of seizures.
Adolescent↗
Endothelial cell surface F1-F0 ATP synthase is active in ATP synthesis and is inhibited by angiostatin.
Angiostatin blocks tumor angiogenesis in vivo, almost certainly through its demonstrated ability to block endothelial cell migration and proliferation. Although the mechanism of angiostatin action remains unknown, identification of F(1)-F(O) ATP synthase as the major angiostatin-binding site on the endothelial cell surface suggests that ATP metabolism may play a role in the angiostatin response. Previous studies noting the presence of F(1) ATP synthase subunits on endothelial cells and certain cancer cells did not determine whether this enzyme was functional in ATP synthesis. We now demonstrate that all components of the F(1) ATP synthase catalytic core are present on the endothelial cell surface, where they colocalize into discrete punctate structures. The surface-associated enzyme is active in ATP synthesis as shown by dual-label TLC and bioluminescence assays. Both ATP synthase and ATPase activities of the enzyme are inhibited by angiostatin as well as by antibodies directed against the alpha- and beta-subunits of ATP synthase in cell-based and biochemical assays. Our data suggest that angiostatin inhibits vascularization by suppression of endothelial-surface ATP metabolism, which, in turn, may regulate vascular physiology by established mechanisms. We now have shown that antibodies directed against subunits of ATP synthase exhibit endothelial cell-inhibitory activities comparable to that of angiostatin, indicating that these antibodies function as angiostatin mimetics.
Adenosine Triphosphate↗