Galantamine for the treatment of BPSD in Thai patients with possible Alzheimer's disease with or without cerebrovascular disease.

OBJECTIVES: This study was to investigate an efficacy of galantamine in treatment of behavioral and psychological symptoms of dementia in Thai elderly who suffered from possible Alzheimer's disease (AD) with or without cerebrovascular disease and vascular dementia. METHODS: A 6-month, multicenter, open-label, uncontrolled trial was undertaken in 75 patients. Eligible patients received an initial galantamine dose of 8 mg/dayand escalated over 5 to 8 weeks to maintenance doses of 16 or 24 mg/day. The behavioral response was assessed as an intention-to-treat analysis using the Behavioral Pathology in Alzheimer's Disease Rating Scale (BEHAVE-AD). RESULTS: Galantamine improved behavioral and psychological symptoms of dementia (P < .05 vs baseline) over the 24 weeks of treatment. BEHAVE-AD score was significantly improved from baseline in paranoid and delusion ideation, diurnal rhythm disturbances, anxieties, and phobias. CONCLUSIONS: Galantamine may be a well-tolerated and effective treatment option for improving psychotic, behavioral, and psychological symptoms in Thai elderly with possible AD with or without cerebrovascular disease and vascular dementia.

State of the art: deep brain stimulation in Parkinson's disease.

Surgical therapy for Parkinson's disease has a long history beginning in the 1930s with empirical exploration of different brain targets, such as resection of the primary motor cortex or extirpation of the caudate. Recently, there has been a renaissance of functional neurosurgery for the treatment of advanced Parkinson's disease, particularly deep brain stimulation (DBS). To date, DBS of the globus pallidus interna and subthalamic nucleus has been reported to relieve motor symptoms and levodopa-induced dyskinesia in patients with advanced Parkinson's disease. DBS also has different advantages over pallidotomy and subthalamotomy, including reversibility, decreased risk of reoperation and decreased morbidity. In addition to well-experienced neurologists and neurosurgeons, a multidisciplinary team approach is fundamental and critical to ensure success in the DBS procedure in individual patients. With the advances in neuroimaging, neurophysiology and localization techniques, it is increasingly likely that there will be more surgical targets in the future that can also improve cardinal features of Parkinson's disease, or even nonmotor manifestations of this condition.

Efficacy study of galantamine in possible Alzheimer's disease with or without cerebrovascular disease and vascular dementia in Thai patients: a slow-titration regimen.

The objective is to evaluate the efficacy of galantamine when a slow titration regimen is employed in Thai Alzheimer's disease (AD) patients with or without cerebrovascular disease and vascular dementia (VaD). A 6-month, multicentre, open-label, uncontrolled trial was undertaken in 75 AD patients. Eligible patients received an initial galantamine dose of 8 mg/day and escalated over 5-8 weeks to maintenance doses of 16 or 24 mg/day. Primary efficacy measures were AD Assessment Scale-cognitive subscale (ADAS-cog) and the Clinician's Interview-Based Impression of Change-Plus version (CIBIC-plus). The Behavioural Pathology in AD Rating Scale (BEHAVE AD), the AD Cooperative Study Activities of Daily Living Inventory and Pittsburgh Sleep Quality Index were the secondary efficacy variables. Analyses were based on the intent-to-treat population. Treatment with galantamine showed significant improvement in cognition on the ADAS-cog and CIBIC-plus at month 6. Galantamine showed favourable effects on activities of daily living. Behavioural symptoms and sleep quality were also significantly improved (p < 0.05). Galantamine was well tolerated. The adverse events were mild-to-moderate intensity. The most frequent adverse events commonly reported were nausea (16.4%), dizziness (9.6%) and vomiting (6.8%). The results of this study may be consistent with galantamine being an effective and safe treatment for mild-to-moderate AD patients with or without cerebrovascular disease and VaD. Flexible dose escalation of galantamine was well tolerated. The daily maintenance dose of galantamine was 16 mg/day, followed by a back up dose of 24 mg/day.

Thrombolytic treatment for acute ischemic stroke: a 2 year-experience at King Chulalongkorn Memorial Hospital.

We studied 9 stroke patients who received a thrombolytic agent at King Chulalongkorn Memorial Hospital. Six presented with stroke in the middle cerebral artery territory and three had basilar stroke. Seven patients were given intravenous thrombolysis and 2 received intra-arterial treatment. We strictly followed the inclusion and exclusion criteria for intravenous tissue plasminogen activator (rt-PA) according to the National Institute of Neurological Disorders and Stroke (NINDS) rt-PA study. For patients receiving intraarterial thrombolysis, emergency angiograms were performed. Two patients with severe basilar stroke dramatically improved after intravenous thrombolysis and had very good outcome. Four patients with middle cerebral artery stroke became worse within 24 hours. Three of them died in the acute phase due to intracerebral hemorrhage (2 cases) and massive infarction with brain herniation (1 case). For intra-arterial treatment, good recanalization was seen but clinical improvement was insignificant. The result of thrombolytic treatment in this study was not so impressive, partly because we only treated the very severe cases. The efficacy of this treatment among our population needs to be further investigated.

Magnetic resonance spectroscopy of the brain in neurologically asymptomatic HIV-infected patients.

The CNS involvement is frequently found in human immunodeficiency virus (HIV) infection. The purpose of our study was to determine whether proton magnetic resonance spectroscopy (MRS) could detect early brain involvement in neurologically asymptomatic HIV-infected patients with normal MR imagings and to find the correlation between MRS and the immune status. We performed MRS in 30 HIV seropositive neurologically asymptomatic patients with normal MRI and compared the MRS findings with 13 controls. A statistically significant reduction in N-acetylaspartate (NAA)/creatine (Cr) and N-acetylaspartate (NAA)/choline (Cho) in both centrum semiovale (p < 0.005) and thalamic areas (p < 0.05) was found. There is no statistically significant difference as to choline (Cho)/creatine (Cr) and myoinositol (mI)/creatine (Cr) ratios in both regions. The difference of NAA/Cr was more pronounced in the white matter than in the gray matter. As for the immune status, there was a trend towards correlation between CD4 counts and NAA/Cr but devoid of statistical significance. Our results suggest that MRS is more sensitive than conventional MR imaging in detecting CNS involvement in neurologically asymptomatic HIV patients and may, therefore, be used for early detection of brain damage induced by HIV.

The Thai version of Aachen aphasia test (THAI-AAT).

The lack of a standardized Thai Language aphasia test raises difficulties not only with the assessment and treatment planning for the clinical but also with the accurate diagnosis and the reliable incidence for research on aphasiology in Thailand. This study aimed to use the Thai version of German Aachen aphasia (THAI-AAT), which is systematically adapted according to well-defined linguistic criteria and psychometric requirement, to assess the language deficit of Thai aphasic patients. The subjects participating in this study were 125 aphasia patients, 60 non-aphasic brain damaged patients and 120 normal subjects. The result revealed that the THAI-AAT is linguistically parallel in test design and fulfills the same psychometric properties as the original. The THAI-AAT obtains the goals: to differential diagnosis of aphasia distinguishing it from non-aphasic disturbance and to identify the type of aphasic syndrome.

Comparison of transcranial Doppler ultrasound and computed tomography angiography in symptomatic middle cerebral artery stenosis.

Transcranial Doppler ultrasound (TCD) and computed tomography angiography (CTA) of 10 patients with middle cerebral artery territory stroke were studied. To obtain data from patients with presumed in situ middle cerebral artery (MCA) stenosis, the study excluded patients with a known source of cardiac emboli, significant carotid stenosis and classical lacunar syndrome. As the gold standard for this study, CTA demonstrated MCA stenosis in all patients (100%), while abnormal TCDs suggesting MCA stenoses were found in only six patients (60%). The stenotic sites differed among patients with normal and abnormal TCDs. Patients with false negative TCDs were found to have more distal lesions (distal M1 or M2 segment) whereas patients with TCD abnormalities tend to have more proximal lesions as demonstrated by CTA. It is concluded that an abnormal TCD is highly suggestive of stenosis of MCA. A normal TCD, however, does not exclude such a lesion, especially in patients with distal M1 or M2 stenoses. Therefore, TCD may not be the best screening test for intracranial vascular stenotic lesion in MCA territory stroke.

Multiple cranial neuropathy in Cogan's syndrome.

A 20 year old woman presented with recurrent alternative keratitis for four months. One month before admission, she developed progressive hearing loss, visual impairment, facial diparesis and bilateral trigeminal neuropathy. Cogan's syndrome was diagnosed. Prompt treatment with corticosteroid resulted in dramatic improvement of the ocular, otological and neurological dysfunctions.

Somatosensory evoked potentials in X-linked recessive bulbospinal neuronopathy: a case demonstration.

Clinicopathological findings in X-linked recessive bulbospinal neuronopathy were characterized by loss of myelinated fibers in the fasciculus gracilis and depletion of neurons in the ventral horn throughout the same segments. Clinical profile of this rare motor neuron disease include sign and symptom of lower motor neuron involving bulbar and spinal level with minimal or no sensory deficit. Previous electrodiagnostic findings consist of electrophysiological evidence of anterior horn cell disease and decreased or absent sensory action potentials in the peripheral nerve. The role of somatosensory evoked potential which can uncover the involvement of posterior column has never been probed. We report a 22-year-old man who had a clinical syndrome of X-linked bulbospinal neuronopathy. The peripheral electrodiagnostic studies supported the evidence of prolonged anterior horn cell disease and decreased sensory response. The median SEPs revealed delayed N11-N13 and N13-N20 interpeak latencies representing demyelination in fasciculus gracilis of upper cervical cord. Therefore, the median SEPs, an uninvasive procedure, can be used as a supportive method to identify sensory neuronopathy with posterior column lesion in this syndrome, especially when the patient has no obvious sensory and endocrine symptom.

Syndrome of progressive ataxia and palatal myoclonus: a case report.

A 46-year old man presented with progressive cerebellar ataxia for 5 years. Physical examination revealed palatal and tongue myoclonus, cerebellar gait, limb ataxia and spasticity of the lower extremities. The imaging studies including CT-scan and MRI of the brain revealed progressive pancerebellar atrophy and bilateral hypertrophic degeneration of inferior olives. The clinical course was slowly progressive. Various medications included anticonvulsants, benzodiazepines and antispasticity failed to abolish the abnormal palatal movement and ataxic syndrome. The syndrome of progressive ataxia and palatal myoclonus is a rare and unique neurodegenerative syndrome. The pathogenesis and treatment are still unknown.

Pentazocine-induced fibrous myopathy and localized neuropathy.

A 47 year-old woman who had a 4-year history of intramuscular pentazocine injections in the lower extremities, developed gradual stiffness and weakness of the lower extremities. The thigh and buttock muscles were "wooden-hard" on palpation. The skin was hard, shiny and hairless. Associated clinical and electrophysiological polyradiculopathy and multiple mononeuropathy of the lower extremities were observed. Imaging studies showed calcification and fibrosis of the involved muscles. Muscle biopsy revealed fibrous myopathy. Caution in longterm usage and early recognition of pentazocine toxicity as a neuromuscular complication are important in order to prevent irreversible drug-induced fibrous myopathy and localized neuropathy.

The Thai version of the German Aachen Aphasia Test (AAT): description of the test and performance in normal subjects.

The Aachen Aphasia Test (AAT), originally developed as a test for aphasia language disorders in Germany, consists of six spontaneous speech rating scales and five subtests: Token Test, Repetition, Written Language, Confronting Naming and Comprehension. The study aimed to describe the linguistic properties of the AAT Thai version and to investigate the test performances of the normal subjects. In this study some problems of linguistic changes in the construction of the Thai version were discussed. The results revealed that the normal subjects' performances on the test were independent of age, sex and education level. Therefore, the Thai version of AAT is applicable to the differential diagnosis of the communicative abilities of Thai aphasic patients.

Spontaneous carotid dissection.

Spontaneous internal carotid dissection typically occurs in young or middle-aged patients. It is associated with a high rate of recovery and low mortality. Anticoagulant and antiplatelet drugs fail to demonstrate a significant improvement when compared with placebo. A 38 year-old woman with spontaneous dissection of the internal carotid artery diagnosed by MRI is reported. She had an excellent recovery without treatment and no recurrent attack occurred after one year of follow-up. The clinical manifestations, diagnostic tests and treatment of spontaneous internal carotid disection are briefly reviewed in this communication.

Familial paroxysmal dyskinesia.

Familial paroxysmal dyskinesia is characterized by recurrent episodic dystonia and/or choreoathetosis with totally quiescent intervening periods. It is an autosomal dominant with variable penetrance basal ganglia disorder. An 11 year old girl who presented with brief kinesigenic paroxysmal dyskinesia is reported. The abnormal movements were dramatically controlled by diphenylhydantoin. Spontaneous remission was seen in the elder sister of this family.

Progressive cerebral occlusive disease after hypothalamic astrocytoma radiation therapy.

An 18 year-old woman received radiation therapy for hypothalamic astrocytoma at the age of 11 years. She developed progressive cerebral occlusive vascular disease with moyamoya vessels formation in both carotid systems. Apart from diabetes mellitus, she had no other risk factors for occlusive cerebrovascular disease. The site of occlusion was confined to the field of radiation and the development of moyamoya vessels strongly suggestive of a radiation-induced cause. Radiation therapy around the sella and parasellar region appears to be the most common risk factor for this vasculopathy. Progressive irradiation-induced cerebral vasculopathy is due to accelerated atherosclerosis.

Prevalence and clinical features of chronic daily headache in a headache clinic.

Although chronic daily headache is regarded as a syndrome encountered in headache clinics, clinical characteristics have only rarely been studied and the condition has not been documented in Thailand. To investigate the prevalence as well as clinical features of chronic daily headache in Thai patients, 220 patients visiting Chulalongkorn Headache Clinic were examined. Sixty cases (27.3%) were diagnosed as suffering from chronic daily headache (male to female ratio, 1:5.7). The average age of these patients was 32.7 +/- 9.6 years. Based on the International Headache Society (IHS) criteria, 30% of patients with chronic daily headache could be diagnosed as suffering from migraine and 36.7% from chronic tension-type headache, whereas the remainder had combined features of both headache types and were not classifiable. Diffuse steady pain was the most common headache type reported (65%), however, associated features characteristic of migraine were often noted. These included photophobia (70%), phonophobia (56.7%) and nausea (43%). Thirty-four cases (56.7%) reported that their headache could be aggravated by stress. Daily use of analgesics was reported in 58.3% of cases. We concluded that chronic daily headache is a common problem. Although the mechanism has not been fully clarified, the prevalence of associated psychological factors and analgesic overuse imply their involvement in the pathogenesis of this condition. The criteria of the IHS are not entirely suitable for diagnosis and classification of this disorder, and modification of this classification system is needed.

Idiopathic hypertrophic cranial pachymeningitis: an unusual cause of subacute and chronic headache.

Three cases of idiopathic hypertrophic cranial pachymeningitis are presented. The diagnosis was based on the CT scan or MRI findings (or both) of thickened enhancing dura. In all cases, meningeal biopsies were performed and microscopic findings were compatible with nonspecific inflammation. All cases presented with subacute and chronic localized headache. Two cases had associated chronic meningitis. One cases presented with a syndrome of multiple cranial nerve involvement (polyneuritis cranialis). Corticosteroids, in the form of prednisolone 60 mg/day, were effective in all cases. Two cases with less severe pachymeningitis received corticosteroids for 2 weeks, then were tapered off in 4 to 6 weeks. A case with extensive lesions needed a long-term low dosage of prednisolone, 5 to 10 mg/day for maintenance therapy. Idiopathic hypertrophic cranial pachymeningitis may be related to the Tolosa-Hunt syndrome, the syndrome of polyneuritis cranialis, and multifocal fibrosclerosis.

Chronic progressive external ophthalmoplegia.

Two cases of chronic progressive external ophthalmoplegia were described. Both of them presented with progressive bilateral ptosis and gradual impairment of ocular mobility. One of the patients had abnormal cerebrospinal fluid protein level. Another patient had muscle biopsy which was compatible with mitochondrial myopathy. Other possible causes of chronic progressive external ophthalmoplegia had been excluded by appropriate investigations. Chronic progressive external ophthalmoplegia is now considered as one type of mitochondrial diseases. Missed diagnosis of this syndrome is common in clinical practice.

Colchicine-induced myopathy in renal failure.

A 60 year-old woman with chronic renal failure developed acute proximal muscle weakness after receiving a regular dosage of colchicine. Elevation of muscle enzymes and electromyography were compatible with myopathy. Muscle biopsy revealed variation in muscle fiber size and few vacuolated fibers which were features of colchicine-induced myopathy. The clinical improvement and decreasing in muscle enzyme level occurred after colchicine withdrawal. Other potential causes of myopathy such as chronic renal failure and other drugs were ruled out. We suggested that colchicine should be used with caution in the presence of renal failure especially when other drugs which affect the metabolism of colchicine are also prescribed.

Sjogren-Larsson syndrome.

A 9-year-old boy with classical features of Sjogren-Larsson syndrome was reported. He had had diffuse dry skin with ichthyotic scales since birth. The skin biopsy was compatible with ichthyosis. At the age of two he was unable to walk normally and also had learning problems. Neurological examination revealed spastic paraparesis, macular degeneration and mild mental retardation. MRI of the brain and spinal cord revealed subcortical white matter and corpus callosal lesions as well as focal cerebral atrophy and diffuse spinal cord atrophy.