A transformation of trigeminal neuralgia into SUNCT/SUNA: A case report and literature review / Uma transformação da neuralgia do trigêmeo em SUNCT/SUNA: relato de caso e revisão da literatura

Introduction: Trigeminal neuralgia and Short-lasting Unilateral Neuralgiform Headache with Conjunctival injection and Tearing (SUNCT)/Short-lasting Unilateral Neuralgiform Headache Attacks with Cranial Autonomic Symptoms (SUNA) are characterized by similar clinical manifestations, which may lead to diagnostic confusion. However, the transformation of trigeminal neuralgia into SUNCT/SUNA is a rare phenomenon. This report describes a case of trigeminal neuralgia transformation into SUNCT/SUNA due to neurovascular compression and reviews all previously published cases of trigeminal neuralgia to SUNCT/SUNA transformation in the literature. Case presentation: A 49-year-old Thai male patient presented with progressive right facial pain for a period of three months. One year prior, he developed trigeminal neuralgia along the maxillary branch of the trigeminal nerve, characterized by electrical shock-like pain in the right upper molar, exacerbated by eating. His symptoms were effectively managed with carbamazepine. Nine months later, he began experiencing recurrent electrical shock-like pain along the ophthalmic division of the right trigeminal nerve, accompanied by lacrimation, which failed to respond to continued treatment with carbamazepine. Three months prior to presentation, his symptoms evolved into SUNCT/SUNA, characterized by electrical shock-like pain in the right periorbital area and conjunctival injection, lacrimation. Neuroimaging revealed high-grade neurovascular compression of the right trigeminal nerve by the right superior cerebellar artery. The patient's symptoms resolved following microvascular decompression. Conclusion: Clinicians should be aware that patients with longer disease duration of trigeminal neuralgia who develop new neuralgic pain in the ophthalmic branch division with mild autonomic symptoms may be at risk for transformation into SUNCT/SUNA.

Introdução: Neuralgia do trigêmeo e Cefaléia neuralgiforme unilateral de curta duração com injeção e lacrimejamento conjuntival (SUNCT)/Crises de cefaléia neuralgiforme unilateral de curta duração com sintomas autonômicos cranianos (SUNA) são caracterizadas por manifestações clínicas semelhantes, o que pode levar à confusão diagnóstica. Contudo, a transformação da neuralgia do trigêmeo em SUNCT/SUNA é um fenômeno raro. Este relato descreve um caso de transformação de neuralgia do trigêmeo em SUNCT/SUNA devido à compressão neurovascular e revisa todos os casos de neuralgia do trigêmeo para transformação SUNCT/SUNA publicados anteriormente na literatura. Apresentação do caso: Paciente tailandês de 49 anos, sexo masculino, apresentou dor facial progressiva à direita há três meses. Há um ano, ele desenvolveu neuralgia do trigêmeo ao longo do ramo maxilar do nervo trigêmeo, caracterizada por dor tipo choque elétrico no molar superior direito, exacerbada pela alimentação. Seus sintomas foram controlados de forma eficaz com carbamazepina. Nove meses depois, ele começou a sentir dor recorrente semelhante a choque elétrico ao longo da divisão oftálmica do nervo trigêmeo direito, acompanhada de lacrimejamento, que não respondeu ao tratamento continuado com carbamazepina. Três meses antes da apresentação, os sintomas evoluíram para SUNCT/SUNA, caracterizado por dor tipo choque elétrico na região periorbital direita e injeção conjuntival, lacrimejamento. A neuroimagem revelou compressão neurovascular de alto grau do nervo trigêmeo direito pela artéria cerebelar superior direita. Os sintomas do paciente foram resolvidos após descompressão microvascular. Conclusão: Os médicos devem estar cientes de que pacientes com maior duração da neuralgia do trigêmeo que desenvolvem nova dor nevrálgica na divisão do ramo oftálmico com sintomas autonômicos leves podem estar em risco de transformação em SUNCT/SUNA.

Plasmapheresis or intravenous immunoglobulin for myasthenia gravis crisis in King Chulalongkorn Memorial Hospital.

BACKGROUND: Myasthenia gravis (MG) crisis is a life-threatening and unpredictable complication of MG Prognosis of MG crisis has dramatically improved due to modern immunomodulation. However the choice of plasmapheresis or intravenous immunoglobulin (IVIG) is still a controversial issue. OBJECTIVE: Evaluate the efficacy and outcomes of MG crisis treatment with plasmapheresis or IVIG in King Chulalongkorn Memorial Hospital (KCMH) during the past 5 years. MATERIAL AND METHOD: Episodes of MG crisis with respiratory failure were recruited retrospectively from database of KCMH between 2001 and 2006. RESULTS: Thirty-three episodes of MG crisis with respiratory failure from 26 patients (9 males and 17 females) were documented. Plasmapheresis and IVIG were prescribed in 21 and 9 episodes of MG crisis, respectively. There was no statistical significant difference in baseline characteristics between both groups. The mean duration of intubation in plasmapheresis group was 12 +/- 11.1 days and in IVIG group was 10.3 +/- 4.6 days. The mean length of hospital stay (LOS) in plasmapheresis and IVIG were 30.7 +/- 29.6 days and 25.4 +/- 16.2 days, respectively. Hospital acquired pneumonia (HAP) occurred in four episodes (18.2%) in plasmapheresis and 1 episode (11.1%) in IVIG There was no statistical difference in the outcome of both treatment groups. All patients in both groups were well upon discharge. CONCLUSION: MG crisis with respiratory failure was safely managed with either plasmapheresis or IVIG in KCMH. The present study cannot demonstrate any differences in the efficacy of plasmapheresis or IVIG. This may be due to inadequate sample size thus more patients should be recruited for further study.

Miller-Fisher syndrome at King Chulalongkorn Memorial Hospital.

BACKGROUND: Miller-Fisher syndrome (MFS), a variant of Guillain-Barré syndrome (GBS) is a self-limiting demyelinating disease of the peripheral nervous system. Clinical spectrum, natural history, prognosis, and pathogenesis of MFS are not fully documented. OBJECTIVE: Probe the clinical features of MFS in a tertiary center in Thailand and compare its pattern with other Asian countries. MATERIAL AND METHOD: The clinical recordings were searched from databases at King Chulalongkorn Memorial Hospital (KCMH) between 2002 and 2007. Keywords were "Miller-Fisher syndrome" and "Guillain-Barré syndrome". Cases with MFS were recruited The data regarding clinical features, course, treatments, and investigations were reviewed. Comparisons with other large Asian series were demonstrated. RESULTS: Six patients (male:female; 3:3) with MFS were analyzed The incident rate is 7.7% that of GBS. The median age was 54.3 years (range 28 to 73 years). MFS frequently started with diplopia followed by ophthalmoplegia and ataxia. Other clinical symptoms included limb dysesthesia and weakness, dysphagia, dysarthria, and diffuse headache. Spontaneous recovery occurred in three patients while two patients received plasmapheresis and one received intravenous immunoglobulin (IVIG). Six months after neurological deficits, all patients were almost free of symptoms and had returned to their normal activities. CONCLUSION: The incidence, clinical features, and prognosis of MFS in KCMH were comparable with the previous studies in other Asian countries. High percentage of limb dysesthesia and optic neuropathy were detected in the present series. Headache was also common among Thai MFS. Immuno-pathogenesis of MFS is well documented but immunomodulatory therapy should be considered only in some cases.

Optic neuritis in a patient with Miller-Fisher syndrome.

Miller-Fisher syndrome (MFS) is considered a variant of Guillain-Barrd syndrome (GBS). The syndrome is characterized by acute onset of gait ataxia, ophthalmoplegia, and areflexia. Conventionally, MFS has been considered exclusively a disease of the peripheral nervous system. However there are occasional reports of central nervous system involvement. Here, a 62 year-old woman who presented with classical features of MFS and progressive bilateral dyschromatopsia and reported visual impairment. Normal MRI of the brain and CSF albumino-cytologic dissociation were observed. CSF oligoclonal IgG pattern indicated a passive transfer of oligoclonal IgG from a systemic inflammatory response. Nerve conduction studies showed slow motor conduction velocity in her extremities. The optic fundi were normal. Visual evoked potentials (VEPs) revealed bilateral optic neuropathy. Marked spontaneous improvement of her syndrome was documented within six weeks. Optic neuritis may be a central nervous system feature that should be recognized as part of the MFS.

The effect of Galantamine on sleep quality in Thai Alzheimer's disease patients.

OBJECTIVE: To study the effect of Galantamine on sleep quality in Thai Alzheimer's disease (AD) patients with or without cerebrovascular disease. MATERIAL AND METHOD: A 6 month, multicenter open-label, uncontrolled trial was undertaken in 75 mild to moderate Alzheimer's disease patients with or without cerebrovascular disease. Eligible patients received a flexible-dose of Galantamine 16 or 24 mg/day for 24 weeks. The Pittsburgh Sleep Quality Index (PSQI) with self-analysis questionnaires were used to evaluate sleep quality. Analyses were based on the intent-to-treat population. RESULTS: Seventy-five eligible patients with mild to moderate Alzheimer's disease with or without cerebrovascular disease (male:female = 32:43, age range 74.5 +/- 0.9) were included and 58 patients (79%) completed the present study. The global PSQI scores showed some improvement over baseline (week 0 = 5.10 +/- 3.08, week 4 = 4.37 +/- 2.48, week 8 = 4.65 +/- 2.71, week 24 = 3.70 +/- 2.12) but were not yet statistical significant. In contrast, most of each component scores such as sleep quality, sleep latency, sleep duration, sleep disturbances, sleep medication, and daytime dysfunction except sleep efficiency, showed significant differences from baseline after week 8. Moreover, there were no significant differences in global PSQI and component scores between mild and moderate stages of Thai AD patients or between men and women patients. CONCLUSION: The result of the present study may be consistent with Galantamine being safe and can maintain good sleep quality for mild to moderate Thai AD patients with or without VaD. Galantamine doses of 16-24 mg/day were well tolerated.

Reversible posterior leukoencephalopathy syndrome: a retrospective study in King Chulalongkorn Memorial Hospital.

BACKGROUND: Reversible posterior leukoencephalopathy (RPLE) or reversible posterior cerebral edema syndrome is a syndrome characterized by transient focal or diffused neurological deficits and reversible neuroimaging changes. RPLE is often associated with hypertension and systemic illness. The classical Magnetic Resonance Imaging (MRl) feature of RPLE is predominately involvement of bilateral posterior cerebral white matter. Due to availability of MRI technology, this entity has been reported more frequently MATERIAL AND METHOD: The clinical recordings were searched from data bases at King Chulalongkorn Memorial Hospital from 2003 to 2005. Keywords were "leukoencephalopathy" and "hypertensive encephalopathy". Neuroimaging criteria for the diagnosis of RPLE were bilateral symmetrical cortical-subcortical white matter lesions predominantly affecting the occipital lobe. The data were reviewed. Cases with RPLE were recruited and analyzed. RESULTS: Nine patients (female:male, 8:1), ranged in age from 17 to 39 years (average, 26 years) were analyzed. Five patients had acute hypertension associated with renal failure, 1 had acute hypertension without renal impairment, 2 had eclampsia and 1 was associated with cyclosporine therapy. Most common clinical symptoms were seizure and cortical blindness. MRI revealed bilateral occipital white matter edema in 7/8 patients while computerized tomography demonstrated this feature in 3/9 patients. Other MRI abnormalities were detected in frontal lobes, parietal lobes, deep grey nuclei, brainstem and cerebellum. The patients were treated with antihypertensive drugs, antiepileptics and withdrawal from immunosuppressive therapy. In 8 patients, the neurological deficits recovered within 2 weeks. The case with cyclosporine therapy had residuals in the form of limb weakness and spasticity. CONCLUSION: RPLE is associated with hypertension, systemic autoimmune diseases, renal impairment, immunosuppressive therapy or eclampsia. The neuroimaging findings reveal characteristic white matter vasogenic edema in occipital lobes as well as other cortical areas and deep grey matter. Good clinical outcomes occur after prompt symptomatic treatment with antihypertensive drugs, antiepileptics or withdrawal from immunosuppressive therapy and repeated neuroimaging may not be necessary.

Neurological manifestation of methyl bromide intoxication.

Methyl bromide is a highly toxic gas with poor olfactory warning properties. It is widely used as insecticidal fumigant for dry foodstuffs and can be toxic to central and peripheral nervous systems. Most neurological manifestations of methyl bromide intoxication occur from inhalation. Acute toxicity characterized by headache, dizziness, abdominal pain, nausea, vomiting and visual disturbances. Tremor, convulsion, unconsciousness and permanent brain damage may occur in severe poisoning. Chronic exposure can cause neuropathy, pyramidal and cerebellar dysfunction, as well as neuropsychiatric disturbances. The first case of methyl bromide intoxication in Thailand has been described. The patient was a 24-year-old man who worked in a warehouse of imported vegetables fumigated with methyl bromide. He presented with unstable gait, vertigo and paresthesia of both feet, for two weeks. He had a history of chronic exposure to methyl bromide for three years. His fourteen co-workers also developed the same symptoms but less in severity. Neurological examination revealed ataxic gait, decreased pain and vibratory sense on both feet, impaired cerebellar signs and hyperactive reflex in all extremities. The serum concentration of methyl bromide was 8.18 mg/dl. Electrophysilogical study was normal. Magnetic resonance imaging of the brain (MRI) revealed bilateral symmetrical lesion of abnormal hypersignal intensity on T2 and fluid-attenuation inversion recovery (FLAIR) sequences at bilateral dentate nuclei of cerebellum and periventricular area of the fourth ventricle. This incident stresses the need for improvement of worker education and safety precautions during all stages of methyl bromide fumigation.

Modified Parkinson's Disease Sleep Scale (MPDSS) in Thai Parkinson's disease patients.

BACKGROUND: Parkinson's Disease Sleep Scale (PDSS) has been recently developed for clinical evaluation of sleep problems in PD. The modified PDSS (MPDSS) has been developed in the presented study. OBJECTIVE: To evaluate the usefulness of MPDSS and sleep problems in Thai PD patients. MATERIAL AND METHOD: A cross sectional study in 50 PD patients was conducted at King Chulalongkorn Memorial Hospital. Sleep problems were assessed by the MPDSS, which has more items to cover more aspects of sleep problems in PD than PDSS. RESULTS: MPDSS showed good reliability (Cronbach's Alpha = 0.842). Some Problems regarding the clinical use of MPDSS in Thai patients were detected especially related to the meaning of visual analogue scale. Self-assessment of sleep in PD patients reveal sleep problems only 58%, while MPDSS probed sleep problems in 76% of PD patients. Nocturnal motor symptoms, snoring, sleep initiation and maintenance problem and nocturia were common in Thai PD patients. Disease staging had significant correlation with MPDSS. Prevalence of sleep disorders and trend of each score in PDSS were similar to previous studies in Western countries. CONCLUSION: MPDSS is useful for screening and evaluation of sleep problems in Thai PD patients. Nevertheless, some modifications, especially the visual analogue scale, may be needed to simplify its practical utility. Prevalence and trend of sleep problems in Thai PD patients were similar to the previous studies in Western countries.

Combination of acute stroke unit and short-term stroke ward with early supported discharge decreases mortality and complications after acute ischemic stroke.

BACKGROUND: The stroke unit has been established as a standard care for stroke. However, it has not been widely established in developing countries due to the lack of understanding and limited resources. OBJECTIVE: To compare the complications and mortality of stroke patients admitted in the stroke unit and short-term ward with those admitted in the general medical ward. MATERIAL AND METHOD: The authors prospectively collected data of acute stroke patients who were admitted after the set up of the stroke unit and stroke short-term ward in 2003, and compared with the data of those who were admitted in a general medical ward in 2001. All acute stroke patients who presented within seven days of the onset were admitted and those who had final diagnosis of ischemic stroke or transient ischemic attack (TIA) were studied. Patients in the stroke unit were taken care of by a multidisciplinary team approach under clinical guidelines and a care map. The short-term ward is a part of the general medical ward and stroke patients were treated by a multidisciplinary team followed by homecare treatment. The endpoints were mortality rate, neurological and medical complications during admissions, and the mean length of stay. RESULTS: Seven hundred and ninety-four patients were studied. Three hundred and eighty-seven patients were admitted in 2001 and 407 patients in 2003. Among patients presented 2003, three hundred and one cases were treated in the acute stroke unit whereas 106 were admitted in the short-term ward. There was no difference in stroke risk factors and stroke subtypes between the two groups, except for dyslipidemia and cigarette smoking, which were more prevalent in patients admitted in 2003. Patients in the stroke unit and the short-term ward had significantly less mortality than those in the general medical ward (8.9 and 2.1%). Overall complications in the stroke unit and the short-term ward were 16.8%, compared to 26% of those admitted into the general medical ward. Significantly less brain edema, hemorrhagic infarction, urinary tract infection, pneumonia, and pressure sore were also observed. The length of hospital stay of the patients admitted in 2001 and 2003 was 11.26 and 8.09 days, respectively. CONCLUSION: Combination of organized acute stroke unit and short-term ward with early supported discharge reduces the mortality and complications of ischemic stroke patients during admission as well as the length of stay when compared to the general medical ward. The present study reassures that the combination is useful for hospitals in developing countries, which have limited number of beds in their stroke units.

Inflammatory markers and conventional atherosclerotic risk factors in acute ischemic stroke: comparative study between vascular disease subtypes.

BACKGROUND: Large vessel atherosclerosis and small vessel disease are two major causes of ischemic stroke. In patients with large vessel disease, the lesions can be located in the extracranial carotid or intracranial arteries. OBJECTIVE: To search for the differences of risk factors and inflammatory markers among patients with each subtype of vascular disease. MATERIAL AND METHOD: Patients with acute ischemic stroke who had large vessel atherosclerosis or small vessel disease were studied. Patients with large vessel atherosclerosis were subdivided into extracranial carotid and intracranial stenosis groups. Blood samples were collected for c-reactive protein, erythrocyte sedimentation rate, hemoglobin A1C fibrinogen, fasting plasma glucose, cholesterol, triglyceride, and low-density and high-density lipoproteins. Risk factors and results of the blood tests between the groups of patients were compared. RESULTS: There were 116 patients in the study. Sixty-three patients had large vessel disease, whereas 53 patients had small vessel disease. More prevalence of diabetes and higher c-reactive protein were significantly found in patients with large vessel disease. C-reactive protein on admission was also higher in patients with extracranial carotid stenosis than those with intracranial stenosis. Serum cholesterol and low-density lipoprotein was significantly higher in patients with intracranial stenosis than those with small vessel extracranial disease. CONCLUSION: Diabetes and higher c-reactive protein on admission were associated with large vessel disease. c-reactive protein was also higher in patients with extracranial carotid stenosis but their cholesterol and low-density lipoprotein were significantly lower than those with intracranial disease.

Encephalitis lethargica like illness: case report and literature review.

Recent studies have revealed that encephalitis lethargica (EL) may not be related to influenza virus infection but more likely to be a post-infectious autoimmune disease. The diagnostic clinical criteria for EL like illness include subacute hypersomnolence and ophthalmoparesis followed by Parkinsonism, oculogyric crisis, neuropsychiatric disorders and central respiratory abnormality. Recently, Magnetic Resonance Imaging (MRI), which depicts hypersignal intensity on T2 weighted, and FLAIR images at midbrain, tegmentum, and basal ganglia, have been very helpful diagnostic tests in EL like illness. Nevertheless, EL like illness has never been reported in Thailand. A 17 year-old man presented with hypersomnolence one week before admission. Physical examination revealed drowsiness and ophthalmoparesis. MRI showed bilateral hypersignal intensity lesions on T2 weighted and FLAIR images at midbrain, basal ganglia and temporal lobes. CSF studies showed normal profiles. CSF-PCR for herpes simplex virus, varicella zoster virus, cytomegalovirus, Epstein-Barr virus, Pan-Enterovirus and Westnile virus were negative. CSF Dengue and Japanese encephalitis virus hemagglutination test were negative. He was treated with intravenous dexamethasone and immunoglobulin. Somnolence and ophthalmoparesis were improved. Two months later, he developed schizophreniform features and Parkinsonism. MRI revealed improvement of midbrain and basal ganglia lesions. CSF studies showed normal CSF profiles while oligoclonal bands were positive. Autoimmune profiles and serological tests for post-streptococcal infection as well as syphilis were negative. Thyroid function tests and serum ceruloplasmin were within normal limits. Levo-Dopa, clonazepam and sodium valproate had been prescribed and the clinical syndrome was gradually improved.

Acquired neuromyotonia (Isaacs' syndrome): a case report with autonomic physiologic studies.

Acquired neuromyotonia (Isaacs' syndrome) is a rare disorder characterized by hyperexcitability of peripheral motor nerves. The cardinal features consist of myokymia, pseudomyotonia and contracture of hands and feet. The diagnosis of Isaacs' syndrome is based on the clinical features and classic electromyographic findings. Serum antibodies against Voltage-Gated Potassium Channels (VGKCs) are detected in some cases. The authors report a 17 year-old man presented with difficulty in walking, writing and respiratory discomfort for 7 months. His body weight had decreased from 120 to 70 kilograms during that period. Physical examination was remarkable for profound sweating. Muscles were in a state of contraction, action myotonia without percussion myotonia, myokymia and carpopedal spasm. Electromyography showed classical neuromyotonic and myokymic discharges. The investigations for conditions associated with Isaacs' syndrome were unrevealing. VGKCs antibody were not performed. Treatment with carbamazepine resulted in substantial improvement of the symptoms within 7 days.

The Thai version of the quality-of-life in epilepsy inventory (QOLIE-31-Thai version): translation, validity and reliability.

OBJECTIVE: To assess the validity and reliability of the QOLIE-31-Thai Version. MATERIAL AND METHOD: The original questionnaire of the QOLIE-31 was first translated into Thai and, then, item comprehension was assessed. Back translation into English and cross-cultural modification were conducted. Its reliability was assessed using a sample of consenting epileptics aged 18-65 years visiting community hospitals in Nakhon Ratchasima Province, Thailand. RESULTS: One hundred and sixty one epileptics completed the questionnaire. The internal consistency of each scale of the QOLIE-31 was above the accepted standard of 0.7, except for Cognitive Functioning, Medication Effect and Social Functioning. CONCLUSION: The QOLIE-31-Thai Version is reliable for use in Thai rural epileptics even for low educated epileptics but interviews might have to be used instead.

Extensive anterior cranial fossa idiopathic hypertrophic pachymeningitis: a case report and review of the literature.

Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory and fibrosing process of unknown etiology. This entity is characterized by fibrosis and thickening of the dura mater and resulting in neurological syndrome. The authors report a 72 year-old woman who presented with progressive bifrontal headache, bilateral visual loss and transient episode of confusion. Neurological examination revealed bilateral optic atrophy, apathy and no focal neurological deficit. Investigations showed anemia of chronic disease, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. No specific inflammatory diseases or malignancy such as systemic lupus erythematosus, syphilis, hematologic malignancy were found MRI of the brain revealed thickened and enhanced dura mater and leptomeninges at the inferior aspect of bilateral frontal lobes as well as vasogenic edema of the frontal lobes. Cerebrospinal fluid showed mild pleocytosis, high protein level and normal glucose level. Meningeal biopsy revealed nonspecific inflammatory process of the dura and leptomeninges. There was no granuloma formation or evidence of vasculitis. Special stain for tuberculous bacilli, fungus and malignancy were all negative. The diagnosis of "idiopathic hypertrophic pachymeningitis" was made. The patient was treated with oral prednisolone 45 mg/day. Her headache was improved, but the profound vision loss in both eyes remained unchanged after 2 years of follow-up. Prednisolone was tapered within 18 months. Idiopathic hypertrophic cranial pachymeningitis usually involves dura at tentorium cerebelli, cavernous sinus and base of the skull. The extensive involvement at the anterior cranial fossa is extremely rare.

Auditory symptoms: a critical clue for diagnosis of MELAS.

Mitochondrial encephalopathy, lactic acidosis with stroke-like episodes (MELAS) is a rare mitochondrial disorder that affects adults. MELAS syndrome can mimic cerebrovascular disease, encephalitis or toxic-metabolic encephalopathy. The authors reported two patients who presented with auditory symptoms before the onset of encephalopathy and stroke-like episodes. The first patient was a 28 year-old man, who presented with acute sensorineural hearing loss (SNHL) followed by headache, left hemiparesis and generalized tonic-clonic seizure. CT scan of the brain showed hypodensity lesion at the tip of right temporooccipital region. Audiogram and brainstem auditory evoked potential (BAEP) showed abnormal conduction of left brainstem auditory pathway. MRI of the brain showed a lesion involving gray and white matters of the right occipital, parietal and temporal lobes. The distribution of the lesions was not compatible with distribution of arterial supply. MRA was normal. The second patient was a 56 year-old woman with a one-year history of hearing loss. The audiogram revealed bilateral SNHL. A few days before admission, her hearing was acutely deteriorated She could not understand a conversation while she could communicate by writing. CT scan of the brain showed hypodensity in both temporal lobes and MRI revealed lesions in the same area. Pure tone audiogram showed moderate SNHL but BAEP was normal. One week later, she developed global dysphasia and generalized tonic-clonic seizure. Both patients had elevated cerebrospinal fluid and serum lactate: pyruvate ratio. Polymerase chain reaction-restriction fragment length polymorphism disclosed A3243G mtDNA mutation in the blood in the first patient and in muscle biopsy in the second patient. Ubiquinone supplement was prescribed The auditory symptoms in combination with stroke-like episode in supratentorium are important clues to diagnose MELAS syndrome.

Tourette's syndrome: old syndrome, new insights and new treatment.

Recognized for over 300 years, Tourette's syndrome was originally ascribed as a rare bizarre psychogenic illness. Because of recent advances in research on Tourette's syndrome, this disorder is not only the rarity once thought, but also a common, biological, genetic disorder with a spectrum of neurobehavioral manifestations that wax and wane during its entire natural course. In addition to standard neuroleptics, much progress in Tourette's syndrome research has widened its pharmacotherapy to include alpha2-adrenergic agonists and atypical neuroleptics as well as behavioral modification, adjustments, and different surgical approaches. Despite a myriad of reports, there are still many unresolved facts, which stimulate research into the underlying mechanisms of this complex neuropsychiatric disorder. We anticipate that continued success of research in this area will lead to molecular insights, identification of vulnerable genes, and eventually novel therapies that can target all aspects of this complex disorder.

Environmental reduplication in a patient with right middle cerebral artery occlusion.

Environmental reduplication or reduplicative paramnesia is one of the content-specific delusions (CSD) which is characterized by reduplication of places. CSD has been reported in focal and diffuse cerebral disorders. A focal lesion such as frontal lobes and the right hemispheric lesion have been documented The authors describe a 66 year-old woman who had a delusion of misidentification for place one month after right middle cerebral artery occlusion. The patient did not have any history of schizophrenia or other psychiatric diseases. The patient believed that her car, furniture and house were duplicated. She also mentioned that her son and friends tried to takeover all of her properties and told everyone that she was insane. The prominent cortical signs were tactile and visual neglect. Neuropsychological assessments revealed poor attention but she had neither confusion nor dementia. Clock drawing and construction tests revealed visuospatial impairment which was compatible with non-dominant hemispheric abnormality. MRI showed evidence of cerebral infarction in the right middle cerebral artery territory. Only one similar patient who had an intracerbral hematoma of the right frontal lobe has been reported in the literature. The role of occipito-parietal and fronto-temporal lobes or their connections in environmental reduplication is proposed.

Hemifacial spasm: results of treatment with low dose botulinum toxin injection.

BACKGROUND: Hemifacial Sapsm (HFS) is a common movement disorder in Thailand. Botulinum toxin type A (BTA) is an effective and safe treatment for this condition. The success of BTA treatment depends on the experience of the clinician. OBJECTIVE: To study the demographic data, efficacy and safety of low dose BTA injection in HFS patients. SETTING: The Spastic and Dystonia Clinic, Department of Rehabilitation Medicine, King Chulalongkorn Memorial Hospital. DESIGN: Open-label, prospective case-series study. PATIENTS: All patients with HFS referred for BTA injection from December 1st, 1995 to November 30th, 2003. METHOD: Sex, age, side of spasm, onset of symptoms before BTA injection, underlying diseases, sites of BTA injection, dose of each BTA treatment, duration of response, efficacy, and side-effects were analyzed. 3-5 units of BOTOX were intramuscularly injected per site to all muscles that had spasm. After injection, a 20-minute cold compression on the first day was followed by 20-minute warm compression with massage at each injection site per day for 14 days. RESULTS : A total of 112 patients with HFS were treated with 874 BTA treatments. There were 71 females (63.4%) and 41 males (36.6%). The mean age was 45 years. 75 patients (67%) were affected on the left side. Mean duration of symptoms was 3.4 years. The sites of injection were orbicularis occuli and orbicularis oris muscles in all 874 treatments (100%). The mean dose of all treatments was 25 units. The mean initial dose was 30.5 units. The mean dose for subsequent injection was 23 units. The mean duration between treatments was 4.7 months. The mean initial duration was 3.5 months. The mean duration for subsequent injection was 4.8 months. The outcomes of treatment assessed at 4 weeks after injection classified as excellent (>80% improvement) were found in 845 treatments (96.7%). Most treatments had no complication (91.9%). Ptosis, facial paresis and double vision were mild and transient, lasting 1-4 weeks. There were no long-term complications of BTA treatment in the present series. CONCLUSION: Low dose BTA injection is an effective treatment for hemifacial spasm patients. There was a longer duration of response in subsequent injections and a lower complication rate in the present study when compared to others.

Nerve conduction studies in chronic arsenic poisoning patients.

OBJECTIVE: To assess the nerve conduction functions among female patients with arsenical dermatoses compared with the controls. DESIGN: Cross-sectional analytic study SUBJECTS AND METHOD: Thirty females with skin lesions consistent with arsenical dermatoses and 27 controls who met the inclusion criteria were investigated by nerve conduction functions. Case findings resulted from a house-to-house survey in village 12, Ronphibun subdistrict and village 5, Saothong subdistrict, Nakhon Si Thammarat Province, southern Thailand in 1995. RESULTS: Differences between the arsenic-exposed population and the reference group regarding nerve conduction velocities (NCVs), proximal and distal latencies and amplitudes of sensory and motor nerve action potentials were not found except for the absent response to the sural nerve stimulation in three subjects of the exposed group. CONCLUSION: The effects of arsenic toxicity on the peripheral nerves in the form of slow nerve conduction velocities were not found among female patients with arsenical dermatoses in Ronphibun. Some patients might have experienced arsenic neuropathy to some degree in the past (before 1987) but they had recovered to some degree at the time of the present investigation (1996) as most of the patients with chronic arsenic poisoning in the present study changed their sources of drinking water from arsenic-contaminated shallow-well water to other sources such as rainwater, tap water or commercial bottled water.

Idiopathic superficial siderosis: a case report.

Superficial siderosis of the central nervous system (SSCN) is a very rare disorder. The clinical syndrome of SSNC consists of sensorineural hearing loss, cerebellar ataxia and myelopathy. The clinical syndrome together with the typical appearance on magnetic resonance imaging (MRI) of hyposignal intensity along the leptomeninges in T2 sequence permit the diagnosis of SSCN. A 58 year-old man who has a history of chronic progressive hearing loss and gait instability for 5 years is presented. The neurological examination revealed bilateral sensorineural hearing loss, cerebellar ataxia and mild spasticity of the lower extremities. MRI showed classical superficial siderosis in the form of hyposignal intensity along the leptomeninges in T2 sequence. The prominent sites of hemosiderin deposition in this case were cerebellar vermis, trigeminal nerves, vestibulocochlear nerves, around the brain stem and spinal cord surface. Cerebrospinal fluid findings confirmed chronic subarachnoid hemorrhage but bleeding site could not be demonstrated. There is no specific treatment available for idiopathic SSCN.