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Biomedical subjects

K Phanthumchinda

Publications and source records attributed to K Phanthumchinda.

At least 37 records · Page 2Linked to original sources

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.

Adolescent↗

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.

Biopsy↗

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.

Adult↗

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.

Adult↗

Superior orbital fissure syndrome as a presenting symptom in hepatocellular carcinoma.

A patient with superior orbital fissure syndrome caused by metastatic hepatocellular carcinoma is reported. She had painful ophthalmoplegia and decreased sensation along the first branch of trigeminal nerve. Histologically confirmation was done by biopsy of sternal metastases. The awareness of various neurological presentations apart from hepatic failure in this malignancy is emphasized.

Adult↗

Huntington's disease in Thailand: a case report.

The first documented case of Huntington's Disease (HD) in Thailand is reported. The patient presented with classical clinical picture, clinical course and family history. Although hereditary chorea is most frequently associated with HD, other hereditary basal ganglion diseases, Syndenham's chorea, and other causes of abnormal movements were considered and excluded by clinical profiles and investigations. Various recent aspects of HD are briefly mentioned.

Adult↗

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.

Adult↗

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.

Adult↗

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.

Adolescent↗

Plasma C3c in immune-mediated neurological diseases: a preliminary report.

Plasma C3c levels were examined in 56 patients with immune (27) and non-immune (29) mediated neurological diseases by crossed immunoelectrophoresis. Plasma samples were collected during the active phase of illness in both groups, usually within 7 days of admission. 11 patients (4 Guillain-Barré Syndrome-GBS, 3 chronic inflammatory demyelinating polyneuropathy-CIDP, 4 myasthenia gravis-MG) had their plasma saved sequentially during the active and the recovery phase. Plasma C3c levels were elevated in the group with immune mediated diseases when compared with those of non-immune mediated diseases. The sensitivity and specificity of C3c as a diagnostic test for immune mediated neurological diseases were 61.4 and 100% respectively with a positive and negative predictive value of 100 and 41%. the C3c levels in plasma correlated well with disease severity in MG and GBS patients. Such a correlation was also evident in all CIDP patients except one that had persistent elevation in the presence of clinical improvement. Results suggest that the plasma C3c level may be useful for differentiating immune from non-immune mediated neurological diseases. Plasma C3c may also be used for monitoring disease severity, particularly in myasthenia gravis.

Autoimmune Diseases↗

Syringomyelia associated with post-meningitic spinal arachnoiditis due to Candida tropicalis.

A 63 year old man who suffered from syringomyelia related to post-meningitic spinal arachnoiditis caused by Candida tropicalis is reported. The clinical syndrome of syringomyelia developed gradually and a definite diagnosis was delayed for more than 10 years. The patient has partially recovered after surgical treatment. This form of fungal infection and its delayed neurological complication in the form of syringomyelia has not been reported previously, to our knowledge.

Arachnoiditis↗

Encephaloradiculopathy: a non-metastatic complication of hepatocellular carcinoma.

We describe a patient with a silent hepatocellular carcinoma in whom a progressive dysfunction developed in the brainstem, cerebrum, and cerebellum. This clinical manifestation was also associated with polyradiculopathy. The changes were microscopically unlike those seen in hepatic encephalopathy, progressive multifocal leukoencephalopathy and other metabolic encephalopathy. The findings seem to have occurred as a paraneoplastic syndrome of the nervous system. Although in the present case, the cause-and-effect relationship between the hepatocellular carcinoma and the encephaloradiculopathy is only hypothetical, we suggest that the demyelinating process which involves both central and peripheral nervous system in our case resulted from interaction of tumor with the host-immunological mechanism. The neurological presentation in our case is unique and has never been reported before.

Adult↗

Superior orbital fissure syndrome as a presenting symptom in hepatocellular carcinoma.

A patient with superior orbital fissure syndrome caused by metastatic hepatocellular carcinoma is reported. She had painful ophthalmoplegia and decreased sensation along the first branch of the trigeminal nerve. Histological confirmation was done by biopsy of sternal metastases. The awareness of various neurological presentations apart from hepatic failure in this malignancy is emphasized.

Adult↗

Successful surgical removal of intrinsic medullar vascular malformation: a case presentation.

A 22-year-old man with an intrinsic arteriovenous malformation (AVM) of the medulla, who presented with recurrent subarachnoid hemorrhage and bulbar palsy was described. The successful total surgical removal of this AVM was demonstrated. The clinical, roentgenographic, pathological features and surgical approach of the brainstem vascular malformation were summarized.

Adult↗

Hereditary essential myoclonus.

A family is reported in which 11 members in 4 generations had a benign form of essential myoclonus of early onset. The myoclonic jerks were mostly asynchronous and dysrhythmic. They occurred spontaneously when relaxed, induced by movement, increased in tonus and emotional stress. Generalized seizures was found in 3 members who also had essential myoclonus. The relationship between essential myoclonus and idiopathic epilepsy was stressed. Beneficial effect was obtained with alcohol and clonazepam.

Adult↗

Spinocerebellar degeneration.

Forty cases with several forms of spinocerebellar degenerations were studied. The diagnosis was based on an appropriate clinical picture, radiological investigation and family history. There are multiple variants of spinocerebellar degeneration and classification of these diseases remains unsatisfactory and therefore controversial. We classified cases into three main groups. The numbers of cases in each of these groups were as follows: cerebellar group = 16 cases, cerebellar plus group = 9 cases, spinal group = 15 cases. The age, age of onset, mode of inheritance, clinical pictures and cerebrospinal fluid analysis of these cases were discussed. Results of various radiological investigations of these cases were reviewed. From our observations we can conclude that the morphologic changes seen in radiological investigations in our cases were more varied than the observed clinical syndromes. The cause of these syndromes remains unknown and there is no established treatment for spinocerebellar degeneration. We can only offer symptomatic treatment, physiotherapy and genetic counselling for this group of patients.

Adult↗

Giant cell arteritis.

An 80-year-old woman was admitted after a 4 months history of severe headache. Laboratory data revealed elevated ESR, mild anemia and alteration of liver function test. Temporal artery biopsy showed the classical picture of giant cell arteritis. The clinical symptoms and laboratory abnormalities reversed to normal after prednisolone therapy. Although the true prevalence of this disease is unknown in Thailand, previous studies in western countries have revealed that it is not a common disease. GCA may be missed or incorrectly diagnosed in elderly patients with headache and a high ESR. Such a diagnosis can subject the patient to complication of GCA, needless biopsy and serious side effects of therapy with large doses of prednisolone.

Aged↗