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PubMed1975

Chronic subdural hematoma in adults. Influence of patient's age on symptoms, signs, and thickness of hematoma.

The relationship of age to clinical and pathological findings was analyzed in 109 adult patients operated on because of chronic subdural hematoma. A well-formed membrane on the inner and outer surface of the hematoma was used as the criterion for chronicity of the hematoma. Younger patients had more evidence of increased intracranial pressure; older patients had more evidence of mental deterioration and pyramidal tract lesions. The interval from trauma to operation was shorter in the young patients. The thickness of the hematoma as measured from angiograms increased with the age of the patient. The cause of this difference is discussed.

Adult↗
PubMed1983

Congenital neural abnormalities presenting with mirror movements in a patient with Klippel-Feil syndrome. Case report.

A young girl with Klippel-Feil syndrome presented with the onset of mirror movements in early childhood. Computerized tomography studies of her cervical spine and brain revealed fibrous diastematomyelia with duplication of the cervical spinal cord and an extra-axial midline posterior fossa cyst, together with the multiple cervical vertebral anomalies. Exploration of the posterior fossa lesion revealed it to be a dermoid cyst. The congenital spinal and cord abnormalities found in this case support the hypothesis that the Klippel-Feil syndrome may be associated with variable duplication of the spinal cord and that mirror movements may be related to impairment of pyramidal tract decussation.

Brain Diseases↗
PubMed1988

Trigeminal schwannoma. Surgical series of 14 cases with review of the literature.

A consecutive series of 14 patients with trigeminal schwannoma managed surgically at the Neurological Institute of New York since 1970 is reported. Nine women and five men (mean age 40 years) were diagnosed following a mean symptom duration of 33 months. Abnormalities of trigeminal nerve function were present in 11 patients on admission examination. Facial pain was a prominent feature in eight patients. Two patients, both with schwannomas arising from the trigeminal root, presented initially with typical trigeminal neuralgia. Additional cranial nerve palsies or cerebellar or pyramidal tract signs were noted in eight patients. The surgical approach to these tumors depends on their anatomical location. Four patients had tumors confined to the middle fossa, three patients had tumors limited to the posterior fossa, and seven patients had both supratentorial and infratentorial components of their tumors. Twenty operative procedures were performed on these patients, resulting in complete extirpation in six patients, nearly complete removal in seven patients, and partial removal in one patient. Adherence of the tumor to the lateral wall of the cavernous sinus or the brain stem precluded total removal. There was one postoperative death. In the immediate postoperative period, abnormalities of cranial nerves controlling the extraocular muscles were common. In general, these deficits were transient; however, some permanent loss of trigeminal nerve function occurred in nine patients. Two patients required tarsorrhaphy for neurotropic keratitis, and two patients underwent cerebrospinal fluid (CSF) shunting procedures for hydrocephalus or for a persistent CSF leak. The follow-up period ranged from 4 to 177 months (mean 47 months). The clinical features, anatomical considerations, and surgical approach to these rare tumors are discussed. A clinical review of 106 additional cases of trigeminal schwannoma, reported in the English literature since 1935, is also presented.

Adolescent↗
PubMed1998

Irving S. Cooper (1922-1985): a pioneer in functional neurosurgery.

Irving S. Cooper (1922-1985), the son of a salesman, worked his way through high school, college, and medical school to become one of the pioneers in functional neurosurgery. He developed several novel techniques for the surgical management of Parkinson's disease and other crippling movement disorders. A keen interest in the physiology of movement disorders was kindled by his doctoral research and continued during his neurosurgical training. He began to apply this knowledge to surgical practice in 1952 when he began his faculty career as Assistant Professor of Surgery at New York University. At the time, surgical treatment of parkinsonian tremor focused on various techniques used to interrupt the pyramidal tract. During a subtemporal approach for a cerebral pedunculotomy, he inadvertently injured and, subsequently, was forced to occlude the anterior choroidal artery. Much to Cooper's surprise, following emergence from anesthesia the patient's tremor and rigidity were abolished without any residual hemiparesis. This serendipitous observation, together with Meyer's earlier work on the role of the basal ganglia in motor control, helped focus surgical efforts on targets within the basal ganglia and, subsequently, within the thalamus to alleviate the movement disorders associated with Parkinson's disease. While at New York University, Cooper developed chemopallidectomy and, later at St. Barnabas Hospital in the Bronx (1954-1977), he used cryothalamectomy as a surgical technique for primary control of tremor in patients with Parkinson's disease. Cooper authored many original papers on surgical techniques and several textbooks on the lives of patients afflicted with Parkinson's disease and other crippling movement disorders. Although considered controversial, this fascinating and complex neurosurgeon made significant contributions to this field.

Cryosurgery↗
PubMed2003

Delineating gray and white matter involvement in brain lesions: three-dimensional alignment of functional magnetic resonance and diffusion-tensor imaging.

OBJECT: The role of functional magnetic resonance (fMR) imaging has become increasingly important in the presurgical mapping of gray matter. Neurosurgical interventions often involve fiber bundles that connect critical functional areas. Recently, diffusion-tensor (DT) imaging has enabled the visualization of fiber bundle direction and integrity, thus providing the ability to delineate clearly white matter from gray matter tissue. The main objective of this study was to improve the presurgical assessment of critical functionality in the vicinity of brain lesions by combining DT and fMR imaging methodologies. METHODS: Twenty patients with various space-occupying brain lesions underwent imaging for presurgical evaluation of motor and/or somatosensory functions. The authors focus on five patients with diverse space-occupying brain lesions. Diffusion tensor-based fiber tracking and fMR imaging activation maps were superimposed in three dimensions to visualize pyramidal tracts corresponding to motor and somatosensory regional activation. CONCLUSIONS: The combination of DT and fMR imaging for presurgical functional brain mapping provides valuable information that cannot be extracted using either method alone. The validity and sensitivity of noninvasive functional mapping for surgical guidance could be improved by considering results obtained with both methods. Furthermore, the use of three-dimensional visualization seems crucial and unique for viewing and understanding the complicated spatial relationship among the lesion, gray matter activation, and white matter fiber bundles.

Adolescent↗
PubMed1993

[A case of superficial siderosis of the central nervous system with total deafness].

Superficial siderosis of the central nervous system (SSCN) in a disease characterized by chronic deposition of hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves. Previously the diagnosis of SSCN could only be made at autopsy or during a neurosurgical procedure. Now, however, a diagnosis of SSCN can be made non-invasively by magnetic resonance imaging (MRI). We present the case of a 50-year-old male with SSCN accompanied by bilateral sensorineural hearing loss which gradually progressed to total deafness over a seven year period. This patient also had associated bilateral caloric weakness with episodes of severe recurrent headaches over the two preceding years. The deafness and gait disturbance, which were his chief complaints, were followed by other neurological manifestations including pyramidal tract signs, anosmia, and ageusia. High-field MRI on T-2 weighted images of the CNS showed diffuse marginal hypointensity of the cerebrum, brain stem, and cerebellum. Atrophy of the cerebellum and brain stem was also apparent. Low signal intensity along the proximal segment of the acoustic nerve and the facial nerve was noted from the cistern to the internal auditory canal. Neither bilateral transtympanic promontory nor round window electrical stimulation elicited any sound sensation. In this case it was thought that the acoustic nerve alone or both the acoustic nerve and the cochlea were affected by this disease. Consequently, cochlear implantation was not indicated. The source of bleeding into the subarachnoid space could not be detected despite thorough examination. This diagnosis will be made only by physicians who are aware of this rare entity and have knowledge of the characteristic clinical pathology.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗
PubMed2006

[Brain and spinal cord magnetic resonance imaging in spastic paraparesis associated to human T-lymphotropic virus].

BACKGROUND: The spastic paraparesis associated to HTLV-1 causes degenerative pyramidal tract lesions of the spinal cord and affects cortical-nuclear connections in the brain. AIM: To report the findings of magnetic resonance imaging in patients with spastic paraparesis. MATERIAL AND METHODS: A magnetic resonance imaging of the brain and spinal cord was performed in 30 patients (24 females), mean age and evolution of 56 and 12 years respectively, with a clinical and virological diagnosis of tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/HAM). RESULTS: No patient had abnormal signals in the spinal cord parenchyma. However, an atrophy of the dorsal segment was observed in 87% of patients. Patients with the highest degree of atrophy showed a higher degree of functional impairment. Eleven patients had spinal cord conus atrophy, associated to neurogenic bladder or impotency. In 80% of patients, hyperintense subcortical white matter images in DP, T2 and Flair, mostly bi frontal, were detected. In half of them, small rounded and isolated images were observed. In the other half, eight or more images, generally larger and occasionally confluent, were found. Ten of 12 patients with confluent brain lesions showed different degrees of cognitive impairment. No patient had lesions in the corpus callosus, periventricular white matter, pons, medulla oblongata or cerebellum. CONCLUSIONS: Most patients with tropical spastic paraparesis have alterations in brain or spinal cord magnetic resonance imaging. The magnetic resonance lesions are concordant with functional impairment. The characteristics of the imaging in TSP/HAM patients can be helpful in the differential diagnosis of patients with paraparesis.

Adult↗
PubMed1976

A family with neurogenic atrophy of the distal muscles of the upper limbs: clinical and electrophysiological studies.

A Chinese family manifested mild neurogenic atrophy of the distal muscles of the upper limbs. None of the affected members had sensory abnormalities, or pyramidal tract or bulbar involvement. The onset of the illness was in the middle of the second decade of life. The muscle atrophy was more severe in the female members. Electromyographic examination of the atrophic muscles showed evidence denervation. One female patient demonstrated slow motor conduction velocity in the right median nerve.

Adolescent↗
PubMed1976

[Strychnine-neuronographic study of orbital cortex projections to structures of the pons varolii].

In adult cats, it was shown that the orbital cortex had direct contra - and ipsilateral projections to oral and caudal reticular nuclei of the pons, to caudal part of the Guden neuclei, to pons covering nucleus, to sulure nucleus, to trigeminal nuclei, and to such conducting formations, as the trapezoid body and pyramidal tract. The orbital gyri connections were mostly ipsilateral and to the caudal part of caudal reticular nucleus of the pons, to oral part of the reticular giganto-cellular nucleus, and to the own pons nuclei. The highest firing rate and amplitude were recorded in the inhibition zone of the reticular formation (the site of overlapping of the oral and caudal reticular nuclei of the pons).

Animals↗
PubMed1976

[Adrenoleukodystrophy. Clinical-pathologic case history and literature review].

This article presents the clinical and pathological history of a 10-year-old boy with adrenoleukodystrophy as well as a tabular survey of 17 additional cases of which the necropsy records were published in the literature. So far as the case of adrenoleukodystrophy directly observed by the authors themselves is concerned, commencement of the disease was in the seventh year of life, with symptoms of Addison's disease and diffuse inflammatory cerebral sclerosis being observed at about the same time. The cerebral disease manifested itself as a progressive pseudobulbar and pyramidal tract symptomatology and as a progressive cerebral psychosyndrome reminiscent of an endocrinal psychosyndrome. Morphologically, the adrenoleukodystrophy was characterized by severe dystrophy of the cortex of the suprarenal gland and the brain are considered the points of action of a still unknown noxa.e suprarenal gland as well as by extensive sudanophilic processes of decomposition in the cerebromedullary substance, putamen, pallidum, and thalamus. Striated cells and cytoplasmic filamentous inclusions observed in the brain and suprarenal glands in a number of cases reported in the literature were not found by the present authors. The cortex of th

Addison Disease↗
PubMed1999

[A case of medial medullary syndrome detected by diffusion-weighted magnetic resonance imaging].

We report an 84-year-old woman with medial medullary syndrome diagnosed by diffusion-weighted magnetic resonance imaging (MRI). She was admitted because of left hemiparesis and hypesthesia. T2-weighted and diffusion-weighted MRI showed a high signal lesion at the right medial medulla oblongata 10 days after the onset. It is well known that diffusion-weighted MRI is useful for detecting supratentrial cerebral ischemic lesions in the extremely acute stage. However, to our knowledge, there have been only a few reports of diffusion-weighted MRI in patients with ischemic stroke of the medulla oblongata. Normal nerve fibers in the direction perpendicular to the motion-probing gradient (MPG) shows a high signal by diffusion-weighted MRI (anisotropy of apparent diffusion cofficient [ADC]). Normal nerve fibers in the pyramidal tract of medulla oblongata also shows a high signal when the MPG pulse is applied in the x and y directions. We solved this problem by using isotropic diffusion-weighted imaging and were able to detect ischemic lesion of medial medullary infarction in the acute phase.

Aged↗
PubMed1999

[A case of superficial siderosis of the central nervous system--findings of the neuro-otological tests and evoked potentials].

We report a 57-year-old woman with superficial siderosis of the central nervous system. On physical examination, sensorineural hearing impairment, cerebellar ataxia, pyramidal tract signs, dysarthria, and neurogenic bladder were seen. Brain and spinal cord MRI (T2 and proton weighted images) revealed cerebellar atrophy and marginal hypointensity of the Sylvian fissure and brain stem, cerebellum and spinal cord. We diagnosed this patient as superficial siderosis because of the clinical course and specific findings on MRI. We consider this case idiopathic superficial siderosis because the source of the bleeding was unknown. Neuro-otological tests and BAEP disclosed retrocochlear deafness. Her central motor conduction time on MEP was delayed. We discussed the pathogenic mechanism of these conditions, and we concluded that we must take notice of myelinopathy in superficial siderosis and that MEP was useful for detecting them.

Audiometry, Evoked Response↗
PubMed1999

[Electrophysiological findings in patients with SCA6].

The major symptom of spinocerebellar ataxia type 6 (SCA6) is progressive cerebellar ataxia. MRI revealed isolated cerebellar atrophy without brainstem and cerebral involvement. Up to the present electrophysiological abnormalities in patients with SCA6 have not been intensively investigated. We performed electrophysiological examination, such as multi-modality evoked potentials, in 10 patients with SCA6. We analyzed the electrophysiological data including the results previously reported. When compared with SCA1, 2 and MJD, specific findings in electrophysiological studies are obscure in SCA6. Existence of subclinical lesions in peripheral nerves, pyramidal tract, auditory pathway, visual pathway, and sensory pathway was suggested in some cases with SCA6. It is important to consider the effect of age, because age at onset is relatively late in SCA6.

Age of Onset↗
PubMed1999

Distribution of glutamic acid decarboxylase mRNA-containing neurons in rat medulla projecting to thoracic spinal cord in relation to monoaminergic brainstem neurons.

Recent neurophysiological work has suggested the existence of monosynaptic gamma-aminobutyric acidergic (GABAergic) projections from the medulla oblongata to sympathetic preganglionic neurons. The purpose of the present study was to identify the possible anatomical location of these neurons. The location of GABAergic neurons with projection to the thoracic spinal cord was studied by using in situ hybridization for both 65-kD and 67-kD isoforms of glutamic acid decarboxylase (GAD) mRNA (GAD-65 and GAD-67, respectively) combined with midthoracic spinal cord injections of the tracer Fast Blue. Tyrosine hydroxylase (TH) or tryptophan hydroxylase immunohistochemistry was combined with GAD mRNA detection and Fast Blue to determine whether any bulbospinal catecholaminergic or serotonergic cell groups in the medulla also are GABAergic. GAD-67 and GAD-65 mRNA-containing neurons had similar distribution patterns in the medulla oblongata, with some areas exhibiting lighter labeling for GAD-65 mRNA. GABAergic bulbospinal neurons were located in the caudal part of the solitary nucleus, the parasolitary nucleus, the vestibular nuclei, the ventral medial medulla, the raphe nuclei, and parapyramidal areas. TH-immunoreactive neurons in the A1, A2, C1, and C2 areas or the area postrema did not contain either GAD-67 or GAD-65 mRNA. GAD mRNA-positive bulbospinal cells were present medial to theA1 and C1 catecholaminergic cell groups, with little or no overlap. Serotonergic neurons positive for GAD mRNAwere found in the parapyramidal area and just dorsal to the pyramidal tract in the raphe magnus. This population included bulbospinal neurons. In conclusion, GABAergic neurons with projections to the thoracic spinal cord exist in a restricted number of medullary nuclei from which inhibitory sympathetic control may originate.

Animals↗
PubMed1999

Clinical profile of multiple sclerosis in Bengal.

Forty five patients of multiple sclerosis diagnosed on the basis of Poser's criteria from West Bengal were studied. The male-female ratio was 1:1.5, mean age of onset 31.83 years in male and 29.11 years in females. The maximum cases were between the 3rd and 4th decade. Definite MS comprised of 60%, while remaining 40% were probable. Visual impairment (53.33%), weakness of limbs (31.11%) and sensory paraesthesia (20%) were the common presenting symptoms whereas pyramidal tract involvement (93.33%) with absent abdominal reflexes (90%) and optic pallor (64.44%) were common signs. Posterior column and spinothalamic sensations were involved in 55% and 51% of cases respectively. Inter-nuclear ophthalmoplegia was present in 6.66% of cases. Pattern of involvement commonly showed three or more sites of lesion. Optico-spinal affection was present in 22.2% of cases. Relapsing and remitting course was found in 48. 91%, relapsing and progressive course in 33.33% and chronic progressive in 17.8%. MRI of brain showed positive results in 16 out of 23 cases. CSF study showed increased positivity in estimation of immunoglobulin level than oligoclonal band. Findings revalidate the disease pattern as being similar to that in other parts of India as well as Asia.

Adult↗
PubMed1999

[Superficial siderosis of the central nervous system].

We report five cases of superficial siderosis of the central nervous system. All patients developed progressive deafness and cerebellar ataxia associated with pyramidal tract signs or mental deterioration. The cerebrospinal fluid examinations usually revealed an elevated protein level, without other abnormalities. Magnetic resonance imaging typically showed a hypointense rim around the cerebral and cerebellar hemispheres, the brainstem and the spinal cord on T2-weighted images. A definite source of bleeding was only found in two patients. The literature on superficial siderosis is reviewed. The etiologies and the pathogenesis are discussed.

Adult↗
PubMed1999

[A clinical study on long-term outcome in patients with syringomyelia associated with Chiari malformation].

To investigate the long-term outcome in patients with syringomyelia associated with Chiari malformation (chronic tonsilar herniation), we investigated the actual factors of the patient's problems by a questionnaire for the patient. Replies to the questionnaires were obtained from 44 patients with syringomyelia who had been treated with expansive suboccipital cranioplasty with dural plasty (with plugging of the central canal in 20 patients). In 25 cases (68%) in whom the motor dysfunction of the upper extremities remained, the symptoms improved in 16 cases (43%) and were unchanged in 9 cases (24%). In 18 cases (64%) in whom the motor dysfunction of the lower extremities remained, the symptoms improved in 10 cases (36%) and were unchanged in 8 cases (29%). In 31 cases (78%) in whom the sensory disturbance remained, the symptoms improved in 19 cases (48%) and were unchanged in 12 cases (30%). The motor dysfunction of the upper extremities persisted significantly in more patients having a duration of illness over 2 years than in patients with those less than 2 years. Sensory disturbance persisted significantly in more patients with a duration of illness over 3 years than in patients with that less than 3 years. These results suggest that firstly, sensory disturbance (disturbance of the posterior horn) is most apt to remain, then motor dysfunction of the upper extremities (disturbance of the anterior horn) and followed by motor dysfunction of the lower extremities (disturbance of the pyramidal tract). We conclude that patients should be treated before having irreversible spinal cord disturbance.

Adolescent↗