Acanthocytosis and spinocerebellar degeneration: a new association?
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Acanthocytes↗
Biomedical subjects
K K Liao
Publications and source records attributed to K K Liao.
Orientation landmarks of endoscopic transaxillary T-2 sympathectomy for palmar hyperhidrosis.
The identification of the T-2 ganglion through a narrow operative viewfield is the greatest challenge in performing endoscopic transaxillary T-2 sympathectomy, especially for a surgeon who is unfamiliar with the technique. The authors describe a simple anatomical method for identifying the T-2 ganglion during the operation, based on a study of 17 adult cadavers. First, a similar clinical procedure was performed along the anterior or middle axillary line via the second to fourth intercostal spaces to measure the aiming angles and intrathoracic depth needed. Second, the regional anatomical structures and their relationship to bilateral T-2 ganglia were delineated. It was discovered that the superior intercostal artery, a branch of the subclavian artery, was an accessible landmark. This small vessel existed in 87.5% of the cadavers studied. It consistently runs lateral to the parallel sympathetic chain at an average distance of 10 mm. Most important is that it can be easily distinguished where it runs across the inner part of the second rib. The authors emphasize that the superior intercostal artery should be a very beneficial landmark for surgical orientation.
Adolescent↗
Polyneuropathy associated with acute monoblastic leukemia: a case report.
Polyneuropathy associated with acute myelocytic leukemia is rare. We report a woman aged 34 years with acute monoblastic leukemia and polyneuropathy in hematology remission. The clinical, electrophysiological and pathological findings revealed acute symmetrical sensorimotor axonal polyneuropathy that differs from previous reports of three cases.
Adult↗
Intravenous immunoglobulin infusion in multifocal demyelinating motor neuropathy: a case report.
The following report is a case of multifocal demyelinating motor neuropathy (MMN) presenting as a gradual development of asymmetric motor weakness without sensory involvement. Electrophysiological studies showed mainly a conduction block with normal or slightly slow nerve conduction velocity. Cerebrospinal fluid (CSF) protein and serum protein electrophoresis were normal, but serum IgM anti-GM1 ganglioside antibody was elevated. The patient had a poor response to steroid, plasmapheresis and chemotherapy with cyclophosphamide, but significant improvement was noted after intravenous immunoglobulin (IVIG) infusion. MMN is a potentially treatable condition which clinically mimics a motor neuron disease; if treatment with steroid, plasmapheresis and cyclophosphamide have failed, IVIG may be effective.
Demyelinating Diseases↗
Midbrain hemorrhage presenting as bilateral ptosis without hemiplegia: a case report.
A 66-year-old man suddenly developed bilateral ptosis after awaking from a nap. He did not experience nausea, vomiting or headache. In the emergency room, high blood pressure was noted. On examination, his consciousness was clear. Ptosis was present bilaterally and worse on the right side. The pupils promptly constricted to light. He could fully adduct his eyes during conjugate gaze movements, but convergence was impaired in the right eye. There was no diplopia or nystagmus. The assessment of the motor and sensory systems revealed no significant findings. Computed tomographic scanning and magnetic resonance imaging of the brain showed a small hematoma in the midbrain. Six months later, ptosis improved; however, the convergence deficit remained.
Blepharoptosis↗
Sympathetic skin response and R-R interval variation in chronic uremic patients.
Sympathetic skin response (SSR) and R-R interval variation (RRIV) were studied in 36 chronic, nondiabetic uremics to compare with their nerve conduction studies (NCS) and clinical dysautonomia. Abnormal SSR was noted in 5 (13.9%) patients, abnormal RRIV in 14 (38.9%), and abnormal NCS in 26 (72.2%). The patients were classified into three groups: group (GP) 1: "normal," n = 21 (58.3%), normal RRIV and SSR; GP 2: "isolated parasympathetic dysfunction," n = 10 (27.8%), abnormal RRIV and normal SSR; and GP 3: "sympathetic sudomotor dysfunction," n = 5 (13.9%), abnormal SSR. A significant difference in age was found among the three groups (GP 3 > GP 2 > GP 1; P < 0.0001, ANOVA). After controlling the age factor, we still noted a tendency toward increasing NCS disturbances (distal latency and nerve conduction velocity of peroneal nerve; P < 0.05, multiple regression analysis) and frequencies of clinical autonomic symptoms (postural dizziness and impotence; P < 0.05, Mantel-Hanszel test) from GP 1 to GP 3. Patients with abnormal SSR (GP 3) displayed significantly higher frequencies of postural dizziness and impotence, indicating the relationship between an absence of SSR and clinical dysautonomia.
Aged↗
Masseter silent period: a study of magnetic stimulation.
We employed magnetic stimulation to study the masseter silent period (SP) in 16 healthy volunteers. Cutaneous perception threshold (CPT or 1 T) was determined. SP threshold was 30% M (maximal output) in each subject, equivalent to 1.5-3 T, and as intensity increased, SP durations prolonged. The correlation was higher with units of % M (r = 0.89) than CPT (r = 0.39). The recommended intensity was 60% M because of least variation of SP durations. Conclusively, magnetic stimulation is a new and painless method to study masseter SP. CPT is less effective in studying masseter SP with magnetic stimulation as the input effectiveness correlates best with % M rather than CPT.
Adult↗
Syncope as the presenting symptom of nasopharyngeal carcinoma.
Syncope is a rare symptom of nasopharyngeal carcinoma, a common tumor in Taiwan. We describe two patients with nasopharyngeal carcinoma who presented with frequent syncopal attacks five months before the diagnosis was made. We postulate that the mechanism of syncope is involvement of the glossopharyngeal or vagal nerve by para-pharyngeal extension of the tumor.
Aged↗
Swallowing difficulty in primary progressive aphasia: a case report.
A 71-year-old right handed man showed insidious and progressive decline of language skills, in contrast with relatively preserved performance on tests and ability to be independent in daily living. Three years later, he developed swallowing difficulties. MRI scans showed widening of the left sylvian fissure. A SPECT scan demonstrated focal hypometabolism restricted to the left temporal lobe. Videofluoroesophagography revealed marked hesitation of oral preparation and oral phases, while the reflex phases were unimpaired. The patient failed to perform oral tasks on command and imitation without motor and sensory deficit. We believe that his swallowing difficulty was due to oral apraxia. This case adds to the heterogeneity of patients with primary progressive aphasia.
Aged↗
Cardiovascular autonomic functions in Alzheimer's disease.
We investigated cardiovascular autonomic functions in 23 patients with Alzheimer's disease (AD) and 23 age-matched control subjects. The vagal parasympathetic function was assessed with R-R interval variation (RRIV), and sympathetic function with sympathetic skin response (SSR) and orthostatic cardiovascular reflexes. Compared with controls, the AD patients had significantly depressed RRIV during rest (6.4 +/- 1.3% vs. 7.5 +/- 1.6%, p < 0.02) and deep breathing (12.6 +/- 4.6% vs. 17.0 +/- 5.1%, p < 0.006), but all AD patients had normal SSR. After standing for 3 minutes from supine, the changes of blood pressure and heart rate from baseline in AD patients did not differ from those in controls. The AD patients had a lower systolic blood pressure than controls in both supine (130.7 +/- 17.4 vs. 145.4 +/- 20.7 mmHg, p < 0.02) and standing (129.2 +/- 20.1 vs. 146.6 +/- 21.1 mmHg, p < 0.008) positions, especially in those patients with more severe dementia. We conclude that AD patients have mildly impaired autonomic functions, mainly in vagal parasympathetic functions. The pathogenesis and clinical significance of low blood pressure in AD needs further study.
Aged↗
Stroke associated with methamphetamine inhalation.
Seven patients (all men, mean age 32 years, range 17-47) with stroke following methamphetamine inhalation were collected during the last 2 years. Like oral or intravenous abusers, our patients had more hemorrhagic (n = 5) than ischemic strokes (n = 2). Cases of intracerebral hemorrhage (ICH) were lobar (n = 3), caudate (n = 1) or putaminal (n = 1), whereas the infarctions were both in the middle cerebral artery region. Each stroke event occurred within 3 days after drug use. Three patients had hypertension on admission. Though young in age, most patients had multiple stroke risk factors. In 3 patients with ICH, we also found small, low attenuated lesions on the brain computed tomography, however, without clinical correlations. Except for arteriovenous malformation in 1 patient, all angiograms failed to show vasculopathy or vasospasm. Contrary to what one might surmise from previously published reports, methamphetamine inhalation is at least as likely to produce ICH as it is to produce brain infarction.
Administration, Inhalation↗
Ultrastructure, ZIO-staining and chromaffinity of gerbil pinealocytes.
The ultrastructure and cytochemistry of the gerbil pineal gland were studied by the conventional electron microscopy, zinc iodide-osmium tetroxide (ZIO) staining and chromaffin reaction. Conventional electron microscopy revealed that the ultrastructure of gerbil pinealocytes are similar to other rodents, i.e., irregular cell contour with numerous cytoplasmic processes, round or oval nucleus and prominent nucleoli, elongated mitochondria with flattened and tubular cristae and dense matrix, well-developed Golgi apparatus and its associated structures, abundant elements of endoplasmic reticulum--both smooth and rough varieties, and bundles of microfilament and microtubule in the cytoplasm. Some pinealocyte processes contain numerous small clear and "slightly coated" vesicles. Numerous profiles of varicosities containing small dense-cored and clear vesicles were frequently encountered. After ZIO treatment, ZIO staining was preferentially localized in the cytoplasm of some, but not all, of the gerbil pinealocytes. Numerous small clear vesicles (30-50 nm in diameter) in the process of the pinealocytes or in the varicosities of the nerve fibers showed strong ZIO-philia. After chromaffin reaction treatment, the number and electron density of small clear and dense-cored vesicles in the profiles of nerve varicosities increased and this indicates that some of the small clear and dense-cored vesicles in the varicosities are reactive. It is thus concluded that (1) the vesicles in the pinealocytes may be rich in cystine and/or cysteine and possibly the organelle is involved in the sequestering calcium ion during the calcification of the pineal concretions, and (2) the small dense-cored and clear vesicles in the nerve fibers in the gerbil pineal parenchyma may contain both serotonin and primary biogenic amines.
Animals↗
Measurement of cervical canal sagittal diameter in Chinese males with cervical spondylotic myelopathy.
BACKGROUND: Cervical canal encroachment can be properly assessed from lateral cervical radiographs either by defining its magnification rate or by a ratio-method. Data of the latter obtained from Chinese males with cervical myelopathy were compared with the data of general population in different age groups. METHODS: A total of 200 Chinese males were included in this study. Half of them had undergone decompressive procedures for cervical myelopathy, while the remaining 100 cases were volunteers. Sagittal diameters of cervical canal and vertebra were measured from C3 to C6 on lateral cervical radiographs, while ratios (Torg-Pavlov's ratios) of the two reflected the extent of sagittal canal encroachment. Cut-off values and discriminant rates of these parameters in different age groups were obtained by discriminant analysis. The tube-to-film distance was set as 40 inches. RESULTS: In myelopathic group (age < 55 y/o), Torg-Pavlov's ratios, as expressed by mean +/- standard deviation, were 0.77 +/- 0.12 for C3, 0.75 +/- 0.14 for C4, 0.80 +/- 0.14 for C5, and 0.81 +/- 0.15 for C6. In control group (age < 55 y/o) the ratios were 0.94 +/- 0.12, 0.95 +/- 0.13, 0.97 +/- 0.13, and 0.97 +/- 0.13 respectively. In myelopathic group (age > or = 55 y/o), the ratios were 0.76 +/- 0.09, 0.71 +/- 0.10, 0.73 +/- 0.11, and 0.76 +/- 0.11; while in control group (age > or = 55 y/o), the ratios were 0.93 +/- 0.10, 0.89 +/- 0.09, 0.88 +/- 0.11, and 0.91 +/- 0.12 respectively. The cutoff values and their discriminant rates are also presented. CONCLUSIONS: The differences of canal sagittal diameter as well as Torg-Pavlov ratio between myelopathic and control group of Chinese males in individual age group were statistically significant. It is concluded that congenitally narrow cervical canal is a major predisposing factor to cervical spondylotic myelopathy.
Adult↗
Evaluation of cervical radiculopathy by cervical root stimulation.
For precise evaluation of cervical radiculopathy, cervical root stimulation (CRS), conventional electromyographic (EMG) studies, nerve conduction velocity (NCV) studies and F responses were done in 32 patients with clinical symptoms and signs of cervical radiculopathy. While performing CRS, a monopolar needle was inserted into the paraspinal muscles, and the compound muscle action potentials (CMAPs) in the biceps, triceps and abductor digiti minimi muscles were recorded. Conventional EMG was abnormal in 18 (56.2%), whereas CRS was abnormal in 25 (78.1%). Among the 25 patients with positive CRS tests, 13 received surgical decompression for cervical root compression caused either by a cervical disc or by foramen narrowing due to spondylosis. Only 10 out of 13 (76.9%) had abnormal conventional EMG findings. CRS provides a sensitive method for making a direct evaluation of proximal root conduction and is a good aid for pre-surgical evaluation of cervical radiculopathy.
Action Potentials↗
Sympathetic skin response and R-R interval variation in Parkinson's disease.
We investigated autonomic function in patients with idiopathic Parkinson's disease (PD) by measuring sympathetic skin response (SSR) and R-R interval variation (RRIV). Sixty-two PD patients and 62 age-matched normal subjects were recruited. Abnormal SSR was noted in nine (14.5%) PD patients, including three in Stage II, three in Stage III, and three in Stage IV, but not in Stage I patients or normal subjects. Four of these nine patients had postural hypotension. Abnormal SSR was correlated with duration of illness and impotence. In PD patients, abnormal SSR may be due to intermediolateral column dysfunction. After logarithmic transformation and age adjustment, 19 (31.6%) of 60 PD patients had abnormal RRIV during rest and deep breathing. Abnormal RRIV was not related to staging or duration of illness. Patients with constipation had significantly lower RRIV, indicating parasympathetic dysfunction. RRIV was not affected by acute or chronic L-dopa treatment. The agreement between RRIV and SSR in PD patients was poor (kappa = -0.07). It appears that abnormal SSR, but not RRIV, may be associated with more autonomic disturbances in PD patients.
Aged↗
Technetium-99m hexamethylpropylene amine oxime single photon emission tomography of the brain in early Parkinson's disease: correlation with dementia and lateralization.
Regional cerebral blood flow was assessed in 19 patients with early idiopathic Parkinson's disease (PD) and 12 control subjects of similar age by single-photon emission tomography using technetium-99m hexamethylpropylene amine oxime (HMPAO). Of the patients with PD, seven were mildly demented and 15 presented with hemiparkinsonism. Mean HMPAO cortical or basal ganglia/cerebellum activity ratios were calculated. Mean cortical and regional uptake ratios in non-demented PD patients were not significantly different from values in the controls. In contrast, besides generalized cortical hypoperfusion, demented PD patients had significantly lower HMPAO uptake in the frontal and basal ganglia regions than non-demented patients. These observations support the hypothesis of impaired neuronal activity in both cortical and subcortical regions of the brain in demented PD patients. In hemiparkinsonian patients, the only asymmetrical finding was a relative hypoperfusion in the contralateral parietal region. This may be due to deafferentation of the thalamoparietal pathways. The lack of asymmetrical uptake in basal ganglia in our PD patients may be explained by their staging at the time of the investigation (stage I and II, Hoehn and Yahr scale).
Aged↗
Lack of cervical paraspinal muscle involvement in juvenile distal spinal muscular atrophy: an electromyographic study on 15 cases.
Electromyography (EMG) of the lower cervical paraspinal muscles was performed in 15 young Chinese males with distal spinal muscular atrophy of the upper extremities. The lack of fibrillation and positive sharp waves in all patients, both in early or active and chronic or steady stages, did not correlate with the EMG status in the affected upper extremity on the same side. This finding is in striking contrast with that in amyotrophic lateral sclerosis.
Adolescent↗
Proximal slowing in carpal tunnel syndrome resulting from either conduction block or retrograde degeneration.
In the carpal tunnel syndrome (CTS), decreased conduction velocity (CV) of the median nerve in the forearm segment has been ascribed to an electrodiagnostic artefact rather than pathophysiological changes. Standard CV of the forearm segment is calculated by subtracting the distal latency, which may not represent an exact assessment of CV in the proximal median nerve. A new technique modified from the method of Stoehr et al. and Pease et al. can exactly measure CV over the forearm. Using this new technique, the forearm nerve action potentials (FNAP) amplitude and forearm nerve conduction velocity (FNCV) proximal to the wrist can be directly determined. Normal subjects and patients with CTS were studied by both the standard and the new FNAP methods. Patients were divided into subgroups according to the severity derived from standard electro-diagnostic findings. By comparing the normal control and patient subgroups, the results show that there was a significant decrease in FNAP amplitudes proportional to severity, but FNCV was reduced to a lesser extent. In addition, the standard forearm median motor CV (MMCV) correlated well with severity, but the reduced MMCV did not correlate with the decreased FNCV. These findings suggest that retrograde degeneration of the median nerve does exist in CTS; however, retrograde degeneration contributes little to the reduced forearm MMCV which substantially results from the block of faster conduction fibres at the wrist. Therefore, technique artefact plays a major role in causing the proximal slowing in the standard electrodiagnosis.
Action Potentials↗