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.

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.

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.

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.

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.

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.

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.

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.

Segawa disease.

A 14-year-old girl with Segawa disease is reported. The dystonic movement began at the age of three and slow progressive deterioration followed. The girl became bed-ridden and required assistance for all activities of daily living. Diurnal fluctuation and sleep benefit was clearly observed. Inheritence appears to be autosomal dominant. A dramatic, immediate and sustained response to L-dopa therapy was noted. This disorder is a form of treatable dystonia in childhood and adolescence and it is recommended that patients in this age group with primary dystonia should be given a trial of low dose L-dopa even though they have a chronic course or severe disability.

Madras pattern of motor neuron disease: case report from Thailand.

A 38-year-old male farmer with classical features of Madras type motor neuron disease was reported. The benign focal atrophy of extremities especially the upper extremities and associated hearing impairment are important clues to the clinical diagnosis. Although there is no pathologic proof of this subgroup of motor neuron disease our data and previous reports strongly suggest anterior horn cell disease and motor nuclei involvement in the brainstem. The nature of the lesion which is responsible for auditory impairment is still controversial.

Neurological manifestations in patients with malignant histiocytosis.

Of the 67 patients who were diagnosed with malignant histiocytosis at the Department of Medicine, Chulalongkorn Hospital, from January 1981 to May 1992, seven (10%) were found to have neurological manifestations. In five patients, neurologic diseases were the presenting clinical features. In the remaining two patients, the disease manifested at the time of relapse. Leptomeningeal infiltration, the most common pattern of involvement, was found in five patients. Involvement of the spinal cord was noted in three patients and cerebral parenchyma in two individuals. Two patients demonstrated peripheral neuropathy. Rapid clinical deterioration was observed in all patients after documentation of neurological diseases with a mean survival of 1.8 months. It is concluded that the frequency and pattern of neurological manifestations in patients with malignant histiocytosis are comparable to patients with non-Hodgkin's lymphoma. However, most patients with malignant histiocytosis manifested the neurological symptoms as an initial presentation while in non-Hodgkin's lymphoma, neurologic diseases usually manifest at the time of relapse or disease progression.

POEMS syndrome: a case with proliferative vasculopathy and a review of cases Thailand.

A Thai male, aged 36 yrs, presented with chronic progressive sensorimotor polyneuropathy and mixed osteosclerotic and lytic lesions at the lumbar vertebrae (L). Generalized hyperpigmentation, bilateral gynecomastia, small testes, hepatomegaly, lymphadenopathy and ascites were observed. Computerized tomography of the lumbosacral spines revealed osteosclerotic changes at L3,4,5 with spiculated bony proliferation at the transverse processes and posterior components of the spines. Histopathological examination of the lesion was compatible with plasmacytoma. Proliferative vasculopathy was demonstrated in plasmacytoma. Serum immunofixation showed an IgM monoclonal gammopathy. Further investigation demonstrated secondary adrenal insufficiency with low level of plasma testosterone. The patient responded well to local radiotherapy and intermittent chemotherapy with melphalan and prednisolone. The histopathological findings of proliferative vasculopathy suggested the important role of capillary leakage as a factor responsible for systemic manifestations in the POEMS syndrome. The reported cases of this syndrome among Thais are summarized.

X-linked recessive bulbospinal neuronopathy.

The first case of X-linked recessive bulbospinal neuronopathy in a Thai family is described. Clinical features of this entity were discussed. Although two members of this family had classical features of X-linked recessive bulbospinal neuronopathy, there were some differences in distribution of weakness among the affected members. This variation among the members of the affected family was firstly described. The clinical features of this disease especially the associated gynecomastia and essential tremors are distinctive and it is possible to diagnose it even in a sporadic case. Although, the prominent feature of the disease is atrophy and fasciculation of muscles, this entity is not a pure anterior horn cell disorder and should be better classified as a multisystem disorder due to multiple organ involvement.

Eales' disease with myelopathy.

The first Thai case of Eales' disease with myelopathy is reported. This entity must be differentiated from other causes of myelopathy such as those due to infectious-inflammatory causes. The ophthalmologic findings are the most important diagnostic clues. Since many infectious diseases such as tuberculosis and dental sepsis may be potentially related to Eales' disease, and these infections are rather common in Thailand, the diagnosis of Eales' disease with neurological complications especially myelopathy should be looked for.

Wilson's disease.

Fourteen cases of Wilson's disease in Thais were studied retrospectively. Most were in the second and third decades. The hepatic form occurs in all age groups and the most common presentation was cirrhosis and complications. Neurological complications were observed during the second decade and consisted of Parkinsonism, dystonic and pseudosclerotic forms. A Keyser-Fleisher ring was detected in 2/3 of the cases. D-penicillamine was the mainstay of our therapy. Death in this series was related to crisis such as acute hemolysis, hepatitis as well as septic complications of cirrhosis. Since Wilson's disease is a treatable and preventable disorder, early clinical diagnosis and screening of asymptomatic siblings is mandatory.