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.

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.

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.

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.

Headache in brain tumor: a cross-sectional study.

Headache is one of the cardinal features of intracranial neoplasm. However, there is little published information on the prevalence and clinical profiles of headache in patients with brain tumor. This cross-sectional study recruited 171 cases of brain tumor. The average age was 39 years (range from 4 to 75 years). The female to male ratio was 1.2:1. The prevalence of headache was 71%. The duration of headache ranged from 3 days to 10 years with an average of 15.7 months. Most prominent headache profiles in this series were intermittent, nocturnal and early morning headache as well as headache upon arising. Most of the patients had mild to moderately severe headache. Headache is more common with tumors below the tentorium cerebelli. It is more prevalent in primary and intracerebral tumor than in metastatic and extracerebral tumor. Headache has lateralizing value, especially in patients with supratentorial lesions who have no obvious increased intracranial pressure.

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.

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.

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.

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.

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.

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.

Subacute combined degeneration of the spinal cord in acute monoblastic leukemia.

A 54-year-old man who had acute monoblastic leukemia and manifestations of subacute combined degeneration (SCD) of the spinal cord is described clinically and pathologically. Infiltration of the wall of the stomach by leukemic cells may produce impairment in gastric absorption of vitamin B12 and leukemia itself may cause deficiency of folic acid. Decreased level of vitamin B12 or folic acid or both may have evoked symptoms of SCD of the spinal cord in this patient.

Increased intracranial pressure associated with spinal cord astrocytoma: a case with CSF dynamic study.

The case of an astrocytoma of the cervical cord associated with signs and symptoms of increased intracranial pressure in a 14-year-old girl is reported. Several possible etiological factors responsible for producing this syndrome are presented. The impairment of CSF absorption at the convexity proven by CSF dynamic study is the main factor for the increased intracranial pressure and the neoplastic inflammatory reaction appears to be the causative mechanism for poor CSF absorption in this patient.

Posttraumatic arachnoid cyst at the cranial base with unusual manifestations.

A posttraumatic epidural arachnoid cyst embedded within the left petrous bone of a 25-year-old woman is reported. The lesion produced clinical manifestations of cerebrospinal fluid rhinorrhea, recurrent meningitides, and neuropathy affecting all divisions of the fifth cranial nerve as well as the eighth cranial nerve on the left side. The location of posttraumatic arachnoid cyst is rare at the cranial base, and the described clinical manifestations are unique.

Serum antibodies to HTLV-I in Thai patients with chronic progressive myelopathy, multiple sclerosis, myopathy and in HIV-seropositive intravenous drug abusers.

Antibodies to HTLV-I were assayed in sera of 9 patients with progressive myelopathy, 11 with multiple sclerosis, 5 with myopathy and in 10 HIV-seropositive intravenous heroin abusers. Clinical features in 9 cases with progressive myelopathy were not different from those previously described in tropical spastic paraparesis associated with HTLV-I infection. No detectable HTLV-1 antibody was found in the sera of any of the 35 patients studied.

Guillain-Barré syndrome and optic neuropathy in acute leukemia.

We report the unique case of an 18-year-old man with acute myelomonoblastic leukemia in whom Guillain-Barré syndrome and optic neuropathy developed as a part of a clinical presentation.