1/28. Osmotic demyelination syndrome with two-phase movement disorders: case report.Osmotic demyelination syndrome (ODS) is characterized by regions of demyelination throughout the brain, which are most prominent in the pons. This demyelinating disease is associated with electrolyte disturbances and typically occurs in patients who are alcoholic or malnourished. movement disorders are not frequently recognized in patients with ODS. This report describes a 22-year-old woman with ODS after correction of profound hyponatremia. The main neurologic symptom was two-phase movement disorder. First, she had acute onset dystonia, then the movement disorder transformed to generalized rigidity and tremors in the delayed second phase. magnetic resonance imaging in the first phase revealed demyelinating lesions in the central pons, bilateral thalami and basal ganglia. In the second phase, the previous myelinolysis had been partially resolved. The clinical course of the two-phase movement disorder did not correlate with the resolving feature of neuroradiologic findings. During the second-phase movement disorder, the patient had a good response to propranolol and trihexyphenidyl.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/28. Primary brainstem injury: benign course and improved survival.Primary brainstem injury following head injury is a rare event. The victims often have features of supratentorial injury, and a primary isolated injury to the brainstem occurring due to shearing stresses or to injury from the tentorial edge is extremely rare. In the presence of supratentorial injury, these patients may have altered sensorium. Isolated brainstem injury may manifest itself as internuclear ophthalmoplegia, anisocoria, rigidity and cerebellar tremor. Such injuries are now being diagnosed more often due to improved imaging techniques. We treated nine such cases who had sustained primary brainstem injury in road traffic accidents, all but one of whom were subsequently independent. Primary brainstem injuries need not be associated with poor prognosis and mortality and may run a benign course with good quality of survival.- - - - - - - - - - ranking = 9keywords = brain (Clic here for more details about this article) |
3/28. Parkinsonism as an initial manifestation of brain tumor.Parkinsonism secondary to neoplasm is uncommon. We report two patients with bilaterally symmetric parkinsonism as the initial presentation of their brain tumors. The first patient was a 71-year-old woman who presented with a gradual onset of bilateral resting tremor, bradykinesia and rigidity. Computerized tomography (CT) of the brain revealed a large parasagittal tumor in the left frontal lobe. The patient completely recovered from the parkinsonian symptoms after removal of the brain tumor. The second patient, a 74-year-old man with a history of renal cell carcinoma of the right kidney suffered from an insidious onset of bilateral bradykinesia, rigidity and gait difficulty. Cerebral metastasis was noted on the brain CT scan. Early recognition of intracranial tumor as the cause of parkinsonism is important for the management of this type of movement disorder. Moreover, brain CT scanning plays an important role in the differential diagnosis of patients with parkinsonian symptoms.- - - - - - - - - - ranking = 9keywords = brain (Clic here for more details about this article) |
4/28. pseudotumor cerebri manifesting as stiff neck and torticollis.Stiff neck and torticollis are significant signs of neurologic disease. Nuchal rigidity is often associated with meningitis, subarachnoid hemorrhage, and posterior fossa tumor. torticollis may be encountered in inflammatory disorders, such as cervical lymphadenitis, or it can be a sign of spinal cord syrinx or of central nervous system neoplasm. We report on three prepubertal children in whom stiff neck and torticollis were the presenting signs of pseudotumor cerebri. In all, the removal of 6-7 mL of cerebrospinal fluid led to prompt relief of symptoms and signs. We suggest that in the presence of unexplained stiff neck or torticollis in children, the optic discs should be examined to exclude pseudotumor cerebri.- - - - - - - - - - ranking = 1.3619259039625keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/28. Progressive encephalomyelitis with rigidity presenting as a stiff-person syndrome.diagnosis criteria of stiff-person syndrome (SPS) include progressive, fluctuating muscular rigidity and spasms with normal neurological examination. The presence of unusual features such as prominent limb rigidity with segmental signs and contracture, evidence of brainstem dysfunction, profound autonomic disturbances, CSF pleiocytosis or MRI abnormalities in patients with SPS presentation allows to classify these patients as progressive encephalomyelitis with rigidity (PER). We report a 50 year-old woman suffering from severe painful spasms of abdominal wall and limb muscles. Neurological examination showed pyramidal signs. EMG disclosed continuous muscle activity with superimposed discharges. Treatment with high doses of diazepam and baclofen led to moderate improvement of generalised stiffness. However, the right arm became more rigid with oedema and vasomotor changes. Subsequently, bilateral nystagmus and internuclear opthalmplegia appeared. There was mild CSF pleiocytosis. Associated auto-immune thyroiditis was found with positive anti-microsome antibodies and decreased thyroid hormones. Search for profound neoplasm was negative. The patient had three subacute bouts then she improved with methylprednisolone. The initial clinical presentation mimicking a SPS with subsequent diffuse involvement of the central nervous system and a striking localisation of a severe rigidity to one arm allowed to suspect the diagnosis of PER. The relationship between SPS and PER remains unclear because of the rarity of these disorders. The observation reported in this paper gives evidence that both the disorders are probably two clinical presentations of the same pathogenic process.- - - - - - - - - - ranking = 2.3619259039625keywords = brain, central nervous system, nervous system (Clic here for more details about this article) |
6/28. dopamine transporter binding in Wilson's disease.INTRODUCTION: In Wilson's disease (WD), brain magnetic resonance images (MRI) show increased signal intensity in T2 weighted images in the lenticular nuclei, thalamus and brainstem, including the substantia nigra. A poor therapeutic response to levodopa in WD suggests the mechanism of a postsynaptic abnormality. However positron emission tomography studies show an involvement of the nigrostriatal presynaptic dopaminergic pathway. CASE REPORT: We report the clinical manifestations in a case of WD with akinetic-rigid syndrome and initial hesitation. The brain MRI showed an increased signal intensity lesion in the substantia nigra region, in addition to basal ganglion and thalamic lesions. However, dopamine transporter (DAT) imaging with 99mTc-TRODAT-1 revealed a nonsignificantly increased DAT uptake, suggesting a normal presynaptic nigrostriatal dopaminergic terminal. CONCLUSION: We suggest that significant heterogeneity can be found in WD patients and a normal presynaptic dopaminergic pathway may occur in some patients, even those with typical akinetic-rigid syndrome and evidence of substantia nigra involvement in the brain on MRI.- - - - - - - - - - ranking = 4keywords = brain (Clic here for more details about this article) |
7/28. Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report.Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/28. Paroxysmal sympathetic storm.INTRODUCTION: Paroxysmal sympathetic storm (PSS) is a rare syndrome characterized by episodic hypertension, hyperhydrosis, hyperthermia, tachycardia, tachypnea, and extensor posturing. case reports: This article describes two cases of PSS: one following traumatic brain injury and the other following cardiac arrest. DISCUSSION: The first responded to labetalol, morphine, and codeine, whereas the second responded to labetalol. CONCLUSION: These observations underscore the importance of central opioid receptors and nonselective beta-adrenergic antagonists in modulating catecholamine pathways.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/28. Sudden death and paroxysmal autonomic dysfunction in stiff-man syndrome.Two women with typical stiff-man syndrome (SMS) developed increasingly frequent attacks of muscle spasms with severe paroxysmal autonomic dysfunctions such as transient hyperpyrexia, diaphoresis, tachypnea, tachycardia, pupillary dilation, and arterial hypertension. autoantibodies to GABA-ergic neurons were identified in the serum of both patients and in the cerebrospinal fluid of one. Both died suddenly and unexpectedly. General autopsy did not reveal the cause of death. Neuropathological studies revealed perivascular gliosis in the spinal cord and brain stem of one patient and lymphocytic perivascular infiltration in the spinal cord, brain stem, and basal ganglia of the other. The occurrence of a chronic inflammatory reaction in one of the two patients supports the idea that an autoimmune disease against GABA-ergic neurons may be involved in SMS. A review of the literature indicates that functional impairment in SMS is severe and prognosis is unpredictable because of the potential for sudden and unexpected death. Both muscular abnormalities and autonomic dysfunctions may result from autoimmunity directed against GABA-ergic neurons.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
10/28. The metabolic landscape of cortico-basal ganglionic degeneration: regional asymmetries studied with positron emission tomography.Regional metabolic rate for glucose (rCMRGlc) was estimated using [18F]fluorodeoxyglucose (FDG) and positron emission tomography (PET) in five patients (four men, one woman; mean age 68; mean disease duration 2.4 years) with clinical findings consistent with the syndrome of cortico-basal ganglionic degeneration (CBGD). Left-right rCMRGlc asymmetry, (L-R)/(L R) x 100, was calculated for 13 grey matter regions and compared with regional metabolic data from 18 normal volunteers and nine patients with asymmetrical Parkinson's disease (PD). In the CBGD group mean metabolic asymmetry values in the thalamus, inferior parietal lobule and hippocampus were greater than those measured in normal control subjects and patients with asymmetrical PD (p less than 0.02). parietal lobe asymmetry of 5% or more was evident in all CBGD patients, whereas in PD patients and normal controls, all regional asymmetry measures were less than 5% in absolute value. Measures of frontal, parietal and hemispheric metabolic asymmetry were found to be positively correlated with asymmetries in thalamic rCMRGlc (p less than 0.05). The presence of cortico-thalamic metabolic asymmetry is consistent with the focal neuropathological changes reported in CBGD brains. Our findings suggest that metabolic asymmetries detected with FDG/PET may support a diagnosis of CBGD in life.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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