OBJECTIVETo study pathologic features of glial cells in progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) and to explore their pathologic significance.

METHODSBrain tissues from 2 cases with PSP and 3 cases with CBD, all confirmed by autopsies, were examined by routine neuropathologic methods, Gallyas-Braak staining and tau immunostaining. Brain tissues from 6 Alzheimer's disease cases, 4 cases with Parkinson's disease and 6 elderly with no neurologic abnormality were used as controls.

RESULTSGallyas-Braak staining demonstrated tuft-shaped astrocytes and coiled-body oligodendroglial cells in the brain tissues of 2 cases with PSP and 3 cases with CBD. The tuft-shaped astrocytes appeared prominently in the frontal and parietal cortex, basal ganglia and grey matter of the brainstem. The coiled-body oligodendroglial cells were distributed widely in the white matter of the frontal and parietal lobes, basal ganglia, brainstem and cerebellum. However, astrocytic plaques, composed of degenerative stubby processes with radiating arrangement, only appeared in the frontal, parietal and cingular cortex, as well as in the striatum of 3 cases with CBD. The astrocytic plaques and tuft-shaped astrocytes coexisted in the same areas, including parietal and cingular cortex and striatum, in CBD. All these glial abnormalities showed tau-positive immunoreaction not found in control cases.

CONCLUSIONSThe tuft-shaped astrocytes and coiled-body oligodendroglial cells are common glial morphologic features of both PSP and CBD. Astrocytic plaques are also characteristically seen in CBD.

Aged ; Aged, 80 and over ; Astrocytes ; pathology ; Basal Ganglia ; pathology ; Brain Stem ; pathology ; Cerebral Cortex ; pathology ; Humans ; Male ; Neurodegenerative Diseases ; pathology ; Oligodendroglia ; pathology ; Supranuclear Palsy, Progressive ; pathology

A previously healthy 16-month-old Korean girl with symptoms of fever, vomiting, and generalized tonic seizure was diagnosed to have Group D non-typhoid Salmonella meningitis. The patient was treated with ceftriaxone (100 mg/kg/day) and amikin (22.5 mg/kg/day) initially and ciprofloxacin (30 mg/kg/day) was added later because of clinical deterioration and disseminated intravascular coagulation. Brain CT performed on the second day showed a well-demarcated low density lesion in the right lentiform nucleus and both caudate nuclei, without evidence of increased intracranial pressure. MRI performed on the 11th day confirmed CT scan findings as well as right subdural fluid collection, brain atrophy, and ventriculomegaly. She underwent subdural drainage and later ventriculo-peritoneal shunt operation. Despite receiving intensive treatment, she still has severe neurologic sequelae. Our case shows that infarctions of basal ganglia and thalami are not specific for tuberculous meningitis and that meningitis complicated by infarction is indicative of grave prognosis.
Basal Ganglia Diseases/radiography ; Basal Ganglia Diseases/pathology ; Basal Ganglia Diseases/complications ; Basal Ganglia Diseases/cerebrospinal fluid ; Brain/radiography ; Brain/pathology ; Case Report ; Cerebral Infarction/radiography ; Cerebral Infarction/pathology ; Cerebral Infarction/complications* ; Cerebral Infarction/cerebrospinal fluid ; Female ; Follow-Up Studies ; Human ; Infant ; Magnetic Resonance Imaging ; Meningitis, Bacterial/radiography ; Meningitis, Bacterial/pathology ; Meningitis, Bacterial/complications* ; Meningitis, Bacterial/cerebrospinal fluid ; Salmonella Infections/complications* ; Tomography, X-Ray Computed/methods

Gitelman's syndrome (GS), also referred to as familial hypokalaemia-hypomagnesaemia syndrome, is an autosomal recessive renal tubular disorder characterised by hypokalaemic metabolic alkalosis, hypomagnesaemia and hypocalciuria. It is caused by a defect of the thiazide-sensitive sodium chloride co-transporter at the distal tubule. This condition was previously confused with Bartter syndrome. Documentation of hypocalciuria helps to differentiate GS from Bartter syndrome. We report a 44-year-old woman who presented with a history of seizure disorder and periodic paralysis. On investigation, she was found to have hypokalaemic metabolic alkalosis, hypomagnesaemia, hypocalciuria, hypoparathyroidism, hypocalcaemia and basal ganglia calcification, consistent with GS. The atypical features in our case, namely basal ganglia calcification and hypocalcaemia, prompted the writing of this case report.
Adult ; Basal Ganglia Diseases ; diagnosis ; pathology ; Brain ; pathology ; Calcinosis ; diagnosis ; pathology ; Diagnosis, Differential ; Female ; Gitelman Syndrome ; diagnosis ; pathology ; Humans ; Hypocalcemia ; diagnosis ; pathology ; Hypokalemic Periodic Paralysis ; diagnosis ; pathology ; Neuroimaging ; Tomography, X-Ray Computed

Cerebrovascular accident (CVA) is the most common cause of neurologic disorder accompanying grave prognosisand its mortality above 50%. Prior to introduction of the CT, the diagnosis have been depended on clinicalfindings and spinal puncture. Radiologic diagnostic methods, such as angiography, ventriculography andradioisotope scanning are invasive and less sentitive in diagnosis of CVA than CT. The size, location andextension of the intracranial pathology and ventricular penetration are accureately and rapidly portrayed by CT.Consequently, CT plays impotant role in effective tratement and evaluation of prognosis in CVA. Authors analyzed63 cases of diagnosed CVA who were performed CT scan in Korea General Hospital from November 1981 to April 1982.The results were as follows. 1. The most prevalent age group of CVA was 6th decade, and then 7th and 5th decadesin decreasing order. The sex ration between male and female was 1.2:1. 2. The causes of CVA were hypertensivehemorrhage (50.8%), vascular occlusive disease(22.2%), anurysm ruture (4.8%), arteriovenous malformation (3.2%)and hemorrhage of unknown etiology (19.0%). 3. The most common site of hemorrhage was basal ganglia (34.6%) andthen thalamus(21.8%) and cerebral lobes(20.5%). In infarction, the common sites were the lobes(64.7%) and thebasal ganglia (35.3%) 4. Round or oval shaped hematomas of high density (85.9%) were frequent findings ofhemorrhage and mass effect occured in 75.6%. 5. All infarctions were low in density ; Most of the lesion wasinhomogeneous(70.6%) and the rests were homogeneous. Mass effects were seen in 29.4%.
Angiography ; Arteriovenous Malformations ; Basal Ganglia ; Diagnosis ; Female ; Ganglia ; Hematoma ; Hemorrhage ; Hospitals, General ; Humans ; Infarction ; Korea ; Male ; Mortality ; Nervous System Diseases ; Pathology ; Prognosis ; Spinal Puncture ; Stroke ; Tomography, X-Ray Computed

OBJECTIVETo explore the clinical and genetic characteristics of patients with dentatorubro-pallidoluysian atrophy (DRPLA).

METHODSDNA analysis for DRPLA gene was performed in two patients. Clinical features and genetic testing of Chinese DRPLA patients reported in the literature were reviewed in terms of initial symptoms, CAG repeat and age of onset.

RESULTSBoth families were confirmed by genetic analysis. In family 1, the number of CAG repeat in the proband, his brother and his mother was determined respectively as 8/65, 8/53 and 8/18. In family 2, the number of CAG repeat was respectively 13/63, 13/18, 18/52 and 13/13 in the proband, his brother, his father and his mother. The size of the expanded CAG repeats has inversely correlated with the age at onset (P<0.05, r=- 0.555). The age at onset of epilepsy was 10 and that for the onset of ataxia is forty years in initial symptom.

CONCLUSIONThe clinical characteristics of DRPLA include epilepsy, ataxia and cognitive impairment. The initial symptoms are epilepsy in adolescence and ataxia in adults. The size of expanded CAG repeats inversely correlates with the age at onset. The initial symptoms are different with different age of onset. It is difficult to diagnose DRPLA at an early stage.

Adolescent ; Adult ; Aged ; Atrophy ; genetics ; Basal Ganglia Diseases ; diagnosis ; genetics ; DNA Mutational Analysis ; Dentate Gyrus ; pathology ; Family Health ; Female ; Globus Pallidus ; pathology ; Humans ; Male ; Middle Aged ; Nerve Tissue Proteins ; genetics ; Pedigree ; Trinucleotide Repeat Expansion ; genetics ; Young Adult

Two major vascular pathologies underlie brain damage in patients with disease of small size penetrating brain arteries and arterioles; 1) thickening of the arterial media and 2) obstruction of the origins of penetrating arteries by parent artery intimal plaques. The media of these small vessels may be thickened by fibrinoid deposition and hypertrophy of smooth muscle and other connective tissue elements that accompanies degenerative changes in patients with hypertension and or diabetes or can contain foreign deposits as in amyloid angiopathy and genetically mediated conditions such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. These pathological changes lead to 2 different pathophysiologies: 1) brain ischemia in regions supplied by the affected arteries. The resultant lesions are deep small infarcts, most often involving the basal ganglia, pons, thalami and cerebral white matter. And 2) leakage of fluid causing edema and later gliosis in white matter tracts. The changes in the media and adventitia effect metalloproteinases and other substances within the matrix of the vessels and lead to abnormal blood/brain barriers in these small vessels. and chronic gliosis and atrophy of cerebral white matter.
Adventitia ; Amyloid ; Arteries ; Arterioles ; Atrophy ; Basal Ganglia ; Brain ; Brain Ischemia ; CADASIL ; Cerebral Amyloid Angiopathy ; Cerebral Small Vessel Diseases ; Connective Tissue ; Edema ; Gliosis ; Humans ; Hypertension ; Hypertrophy ; Metalloproteases ; Muscle, Smooth ; Parents ; Pathology* ; Pons ; Stroke, Lacunar* ; Tunica Media