Case Report: Focal Myoclonus with a Striatal Lesion as a Presentation of Subacute Sclerosing Panencephalitis.

Adult-onset subacute sclerosing panencephalitis (SSPE) is rare, and focal myoclonus as a presenting feature poses a diagnostic dilemma. We report an adult SSPE patient with unusual clinical and radiological features. A 20-year-old girl had jerky neck movement 9 months earlier, which progressed to left hemimyoclonus in 2 months and generalized frequent myoclonus and fall at 4 months. By 6 months, she was bedbound. On examination, her Mini-Mental State Examination score was 10, and patchy retinitis was observed around the macula. Magnetic resonance imaging revealed corpus striatal involvement and electroencephalography showed periodic discharges. Measles cerebrospinal fluid/serum immunoglobulin G index was 3.3 (normal < 1.3), confirming the diagnosis of SSPE. SSPE should also be considered in adults having focal myoclonus with corpus striatal lesion. EEG is helpful in the diagnosis.

Cerebral Venous Sinus Thrombosis From a Tertiary Care Teaching Hospital in India.

OBJECTIVE: The prognosis of cerebral venous sinus thrombosis (CVST) may be dependent on underlying causes and magnetic resonance imaging findings. We report the clinical, laboratory, and radiologic spectrum, and outcome of consecutive patients with CVST. METHODS: Consecutive patients with CVST over the last 15 years were subjected to detailed history, clinical examination, and laboratory tests as per fixed protocol. The diagnosis of CVST was based on MR venography. The magnetic resonance imaging findings and the sinuses involved were noted. The patients were treated with low-molecular-weight heparin or unfractionated heparin for 14 days, followed by oral anticoagulation. Hospital mortality and 3-month outcome (good or poor) were recorded and correlated with various clinical and laboratory parameters. RESULTS: There were 86 patients whose median age was 30 (6 to 76) years; 47 of them were females. Headache was present in 91.9%, vomiting in 68.6%, focal weakness in 53.5%, seizure in 51.2%, and altered sensorium in 53.5%. Female-specific etiology was present in 34%, prothrombotic condition in 62.8%, infections in 19.8%, and malignancy in 2.8%; no cause could be established in 25.6% of patients. Hyperhomocystinemia was present in 45.9% of patients. Five patients died in the unfractionated heparin group. At 3 months, 77 patients were followed up, of whom 68 had complete, 3 had partial, and 6 had poor recovery. The 3-month outcome was related to Glasgow Coma Scale score and type of heparin used. CONCLUSIONS: In our study multiple prothrombotic conditions were common, and low-molecular-weight heparin reduces mortality and results in good outcome.

Worsening of Wilson disease following penicillamine therapy.

BACKGROUND: Penicillamine is a standard therapy for Wilson disease (WD) but some patients have paradoxical worsening. Predictors of such deterioration have not been evaluated. This study documents frequency and predictors of deterioration following treatment in WD. METHODS: 59 consecutive patients with neurologic WD and 4 asymptomatic siblings were prospectively evaluated. Their clinical, laboratory, ultrasound abdomen and cranial MRI findings with and without worsening were compared. Patients were treated with oral penicillamine and/or zinc and followed up at 1, 3 and 6 months or earlier if needed. Deterioration was defined by >10% worsening in baseline Burke-Fahn-Marsden score or appearance of new neurological sign. RESULTS: Patients' median age was 13 years and 13 were females. 19 patients (30.2%) worsened following treatment; 10 within 1 month, 7 in 1-3 months, and 2 after 3 months of treatment. Deterioration was associated with drooling, leukopenia, thrombocytopenia, splenomegaly and evidence of chronic liver disease. None of the asymptomatic patients following zinc therapy deteriorated. CONCLUSIONS: In the deteriorating group, withdrawal of penicillamine resulted in improvement/stabilization in 11 patients, 2 improved by trientine therapy and 4 continued to deteriorate till 3 months. 30.2% patients with WD deteriorated following penicillamine, especially those with chronic liver disease, leukopenia and thrombocytopenia.

ACE and ADD1 gene in extra and intracranial atherosclerosis in ischaemic stroke.

OBJECTIVE: Hypertension is closely linked to ischaemic stroke (IS) and atherosclerosis, but there are no studies correlating the candidate hypertensive gene, namely angiotensin converting enzyme (ACE) and adducin 1 (ADD1) with magnetic resonance angiographic (MRA) abnormality, therefore this study was undertaken. METHODS: Patients with magnetic resonance imaging (MRI) proven IS were included and their demography, stroke risk factors, and clinical findings were noted. Both intracranial (IC) and extracranial (EC) MRA were performed and more than 50% stenosis was considered significant. Angiotensin converting enzyme and ADD1 gene polymorphism was evaluated by polymerase chain reaction (PCR) both in patients and 188 controls. RESULTS: Angiotensin converting enzyme and ADD1 polymorphism were performed in 151 patients whose median age was 60 years and 26.5% were females. Magnetic resonance angiography was abnormal in 77.5%; extracranial magnetic resonance angiography (ECMRA) in 58.3%, and intracranial magnetic resonance angiography (ICMRA) in 66.7%. The conventional risk factors were not different between the IS patients with and without MRA abnormalities. The presence of DD genotype (OR 3.86, 95% CI 0.78-2.28, P = 0·0001) and ADD1 GW genotype (OR 2.05, 95% CI 1.28-3.27, P = 0.003) significantly increased the risk of IS compared to controls. Both genotype and allele frequency of ACE and ADD1 were higher in MRA abnormal IS patients compared to controls; however, these were not significantly different between the patients with and without MRA abnormality. CONCLUSION: In IS patients, DD genotype and D allele of ACE gene and W allele of ADD1 gene were significantly related to MRA abnormality compared to controls but there was no association of ACE and ADD1 gene polymorphism in IS patients with MRA and without abnormality.

Is ß-blocker (atenolol) a preferred antihypertensive in acute intracerebral hemorrhage?

The mortality in intracerebral hemorrhage (ICH) is mainly due to raised intracranial pressure, and its complications mediated by sympathetic overactivity. There is paucity of studies evaluating the role of ß-blockers in the outcome of ICH. This study reports the role of atenolol in reducing mortality, pneumonia, systemic inflammatory response syndrome (SIRS), and 3 months outcome in the patients with hypertensive ICH. 138 consecutive patients with hypertensive ICH were included and their stroke risk factors and clinical details were recorded. Consciousness was assessed by Glasgow Coma Scale and severity of stroke by Canadian Neurological Scale. Volume of hematoma was measures on CT scan and occurrence of SIRS and pneumonia were noted. 3 months outcome was categorized into good (Barthel index >12) and poor (BI < 12). The patients were categorized into those receiving atenolol and nonatenolol. The effects of atenolol on stroke outcome parameters were evaluated. Seventy-nine patients received atenolol and 59 did not and they mainly received amlodipine. There was no difference in the base line clinical characteristics between the two groups except smoking (P = 0.01) and baseline blood pressure (P = 0.007). Atenolol significantly reduced the mortality (11.4 vs 37.3 %, P < 0.0001), SIRS (16.4 vs 40.9 %, P = 0.007), and pneumonia (8.9 vs 30.5 %, P = 0.002) compared to those not receiving atenolol. At 3 months, patients with atenolol had insignificantly better outcome compared to nonatenolol group (49.1 vs 31.9 %, P = 0.11). Use of atenolol in hypertensive ICH results in reduction in mortality, SIRS, and pneumonia which may be due to its ß-adrenergic blocking effect.

Do ACE (rs4646994) and αADDUCIN (rs4961) gene polymorphisms predict the recurrence of hypertensive intracerebral hemorrhage?

This study was undertaken to evaluate the role of ACE and αADDUCIN polymorphisms in patients with recurrent and nonrecurrent hypertensive intracerebral hemorrhage (ICH). A total of 101 nonrecurrent and 33 recurrent hypertensive ICH patients underwent an ACE (rs4646994) and αADDUCIN (rs4961) gene polymorphism study. The risk factors, clinical findings, CT scan abnormalities and functional outcome of recurrent and nonrecurrent ICH were compared. ACE (rs4646994) and αADDUCIN (rs4961) gene polymorphisms were also compared in the two groups and with 198 controls. The patients with recurrent ICH were older compared to those with nonrecurrent ICH and the other stroke risk factors were found in the two groups. Ganglionic-ganglionic pattern of recurrence was the commonest (75.6%) and all had at least one ICH in the location of hypertensive ICH. ACE DD genotype (OR6.18, 95%CI 2.93-13.02) and D allele (OR 2.43, 95%CI 1.70-3.47) were associated with nonrecurrent ICH compared to controls. In patients with recurrent ICH, DD genotype (OR 7.46, 95%CI 2.8-19.4) and D allele (OR 3.16, 95%CI 1.83-5.46) of ACE, and GW (OR 3.49, 95%CI 1.47-8.28), WW (OR 2.9, 95%CI 1.40-4.30) genotypes and W allele (OR 7.46, 95%CI 2.80-19.40) of αADDUCIN were more frequent compared to controls. Recurrent ICH also had higher frequency of WW genotype (OR 9.43, 95%CI 1.49-59.50) and W allele (OR 2.19, 95%CI 1.11-4.03) compared to nonrecurrent ICH. The frequency of DD + WW (P = 0.008) and DD/WW + ID/GW (P = 0.0001) genotypes in the recurrent ICH was higher than in the nonrecurrent ICH and the controls. Variant genotype combinations of ACE and αADDUCIN render the hypertensive patient more vulnerable to recurrent ICH.

Phosphodiesterase 4 D gene polymorphism in relation to intracranial and extracranial atherosclerosis in ischemic stroke.

In ischemic stroke, extracranial MR angiography (ECMRA) is more frequently abnormal in Caucasians and intracranial (ICMRA) in Asians which may have a genetic basis. We report phosphodiesterase (PDE4D) gene polymorphism and its correlation with MRA findings in patients with ischemic stroke. Consecutive patients with MRI proven ischemic stroke undergoing MRA were included in this study. The severity of atherosclerotic stenosis on MRA was categorized into moderate 50%-80%, severe 80%-99%, and total occlusion 100% using NASCET criteria. The polymorphism in SNP 32, SNP 83 and SNP 87 of PDE4D gene was analyzed by PCR both in the patients and in 188 controls. Among the 148 patients, MRA was abnormal in 77% patients; ECMRA in 53.8%, ICMRA in 66% and both were abnormal in 42% patients. The frequency of CC genotype of PDE4D83 was significantly higher in the patients with ischemic stroke compared to controls (OR 3.38, 95% CI 1.61-7.11, P= 0.001). The frequency of TT genotype of PDE4D87 was significantly higher ICMRA abnormalities (20%) compared to normal ICMRA (2%). The genotype and allele frequency of PDE4D83 and PDE4D32 were not significantly related to MRA abnormalities. The role of PDE4D87 in atherosclerosis needs confirmation in larger studies.

Central post stroke pain: clinical, MRI, and SPECT correlation.

OBJECTIVE: The objective of this study was to report clinical spectrum of central post stroke pain (CPSP) and correlate these with magnetic resonance imaging (MRI) and single photon emission computed tomography (SPECT) findings. DESIGN: The study was designed as a prospective study. SETTING: The study was set in a tertiary care teaching hospital. SUBJECT AND METHOD: Twenty-three consecutive CPSP patients were included and their severity of pain, sensory threshold, allodynia, hyperalgesia, and temporal summation were assessed by quantitative sensory testing (QST). Cranial MRI and (99)Tc ethylene cystine dimmer SPECT findings correlated with QST. RESULTS: The duration of CPSP was 5 months (0.25-108). Allodynia was present in 12 patients, punctuate hyperalgesia in 11, and temporal summation in 12. SPECT was abnormal on visual analysis in 17 patients; hypoperfusion in corresponding thalamus in nine, and parietal cortex in 11 patients. Semiquantitative analysis revealed hyperperfusion of thalamus in four and parietal cortex in five patients. MRI revealed infarction in 14 and hematoma in nine patients. The QST findings were similar in thalamic and extrathalamic CPSP. The MRI and SPECT findings were also not different in CPSP patients with and without allodynia. CONCLUSION: The QST findings in patients with CPSP were similar in patients with thalami and extrathalamic lesions. SPECT and MRI findings were also not different in CPSP patients with and without allodynia.

A study of ACE and ADD1 polymorphism in ischemic and hemorrhagic stroke.

BACKGROUND: Stroke is a common cerebrovascular accident. ACE and ADD1 gene are known to be associated with vascular complications leading to stroke susceptibility. The present study was carried out to evaluate the relative frequency of ACE and ADD1 common polymorphisms in ischemic stroke and intracerebral hemorrhage (ICH). METHODS: A total of 386 CT or MRI proven stroke patients were included; 193 each had ischemic stroke and ICH. The locations and type of stroke were noted. ACE- I/D (rs4646994) and ADD1 (rs4961) gene polymorphisms were analyzed by polymerase chain reaction (PCR). The genotype and allele frequencies of ACE and ADD1 polymorphisms were compared between patients and controls as well as between ischemic stroke and ICH. RESULTS: ACE (DD) genotype was significantly higher in ischemic stroke (37.8%) and ICH (33.7%) compared to controls (11.7%). D allele was also more frequent in ischemic stroke (57.3%) and ICH (56.7%) compared to controls (38.3%). ADD1 (WW) genotype and W allele frequencies were not significantly different in ischemic stroke, ICH and controls. In contrast, we found a synergistic role of ACE (DD)*ADD1(GW) interaction showing a positive association in both ischemic and hemorrhagic strokes. CONCLUSION: Our study suggests that ACE (DD) genotype and D allele significantly increase the susceptibility to ischemic and hemorrhagic strokes.

Magnetic resonance angiography findings in patients with ischemic stroke from North India.

BACKGROUND: We sought to evaluate the magnetic resonance (MR) angiography (MRA) findings in patients with ischemic stroke (IS) from North India and correlate the changes with various conventional and nonconventional risk factors. METHODS: The study took place at a tertiary care teaching hospital. The patients with IS were clinically evaluated including body mass index, dietary habits, and family history of stroke. MR imaging, MRA, and testing for blood sugar, lipid profile, B12, folic acid, and homocysteine were carried out. The MRA abnormalities were considered significant if stenosis was 50% or greater and these were categorized into extracranial (EC), intracranial (IC), or combined lesions. The location of infarct on MR imaging was also noted. RESULTS: There were 151 patients whose median age was 60 (22-85) years. The EC MRA was abnormal in 56.3% and the IC MRA in 63.3% of patients, the internal carotid artery being the most common site. Corresponding infarct was present in 64.7% and noncorresponding in 45.3% of patients. The MRA abnormality positively correlated with hypertension and diabetes, and negatively with alcohol consumption. The EC MRA abnormality was more common in upper caste Hindus and Muslims and in the city dwellers. CONCLUSION: In North Indian patients with IS, the frequency of EC and IC MRA abnormality lies between Whites and the Orientals.

Central poststroke pain: a review of pathophysiology and treatment.

BACKGROUND: Central poststroke pain (CPSP) is a disabling morbidity occurring in 8%-14% of patients with stroke. It is infrequently recognized and difficult to manage. OBJECTIVE: We systematically reviewed the pathophysiology and treatment of CPSP. METHODS: We conducted a Medline search using the key words "central post-stroke pain," "post-stroke pain," "CPSP and basic studies," "CPSP and clinical features," "CPSP and pharmacological treatment," "CPSP and nonpharmacological treatment" and "CPSP and treatment guideline." The articles related to CPSP were categorized into clinical features, pathophysiology and treatment, and then systematically reviewed. RESULTS: Stroke along the spinothalamocortical pathway may result in CPSP after a variable period, usually after 1-2 mo. CPSP may be spontaneous or evoked, variable in intensity and quality. It tends to improve with time. CPSP is associated with mild motor symptoms with relative sparing of joint position and vibration sensations. The pathophysiology of CPSP is not well understood, but central disinhibition, imbalance of stimuli and central sensitization have been suggested. There are few class I and class II studies regarding its management. Amitriptyline and lamotrigine (class IIB) are recommended as first-line and mexiletine, fluvoxamine and gabapentin as second-line drugs. In pharmacoresistant patients, repetitive transcranial magnetic stimulation and deep brain stimulation have been beneficial. CONCLUSIONS: CPSP patients present with diverse sensory symptoms and its pathophysiology is still poorly understood. Amitriptyline and lamotrigine are effective treatments. Further studies are needed to understand the pathophysiology and investigate newer therapeutic modalities.

A study of clinical, magnetic resonance imaging, and somatosensory-evoked potential in central post-stroke pain.

UNLABELLED: This study evaluates the clinical spectrum of central post-stroke pain (CPSP) and correlates it with magnetic resonance imaging (MRI) and somatosensory-evoked potential (SEP) changes. Thirty-one consecutive CPSP patients whose median age was 51 years were evaluated and subjected to quantitative sensory testing and median and tibial SEPs. Cranial MRI abnormalities were noted and correlated with clinical and SEP abnormalities. The majority of patients (n = 21) developed CPSP within 3 months of stroke, and CPSP was the presenting symptom in 7 patients. Five patients had focal symptoms and 26 had hemibody symptoms with or without facial involvement. Pain threshold was reduced in 12, and 3 did not have pain perception. Allodynia was present in 11, static in 4, dynamic in 5, and cold in 7 patients. Temporal summation was present in 14, punctate hyperalgesia in 11, and cold hyperalgesia in 3 patients. Cranial MRI revealed infarction in 23 and intracerebral hemorrhage in 8 patients; 16 had thalamic and 15 extrathalamic lesions. SEP was abnormal in 15 of 22 (68.2%) patients. There was no difference in symptoms and severity of CPSP, quantitative sensory testing, and SEP abnormalities in thalamic and extrathalamic stroke. PERSPECTIVE: CPSP is a poorly recognized entity that can interfere with rehabilitation, reduce the quality of life, and interfere with the activities of daily living and recreational activities. This report concludes that the symptoms and severity of CPSP in thalamic and extrathalamic stroke do not differ significantly.