Cases reported "Thalamic Diseases"

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1/92. The stereotactic volumetric information: its role in two-step resection of brainstem and thalamic giant tumor. Report of three cases and technical note.

    BACKGROUND: A compact intracerebral tumoral lesion is usually considered to be completely resectable. Nevertheless, radical resection of a huge lesion located in a critical area may damage the surrounding compressed brain tissue. In cases with a good prognosis, a two-step removal appears to be a safer strategy. methods: In three cases, two with huge brain stem lesions and one with a thalamic lesion, a two-step volumetric stereotactic resection was planned. This strategy allowed us to evaluate the amount of tumor to be removed during the first procedure and to have, during the second operation, an exact definition of the reduced mass with regard to the scar tissue and postoperative adhesions. Furthermore, we avoided significant shifting of the cerebral structures during both procedures. RESULTS: There was a very good final recovery in the cases with brain stem lesions and a minimal deficit in the patient with the thalamic lesion. The patient with a mesencephalic lesion remained comatose for almost 2 days after the first procedure, confirming our fears about too radical a one-step resection. CONCLUSIONS: We think that by using current techniques, it is possible to remove a well circumscribed lesion regardless of its position. This is probably easier with giant lesions where a safe trajectory can be planned. In these cases, with lesions located in very critical areas but with a good prognosis, a two-step resection appears to be a good option.
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2/92. CT and MRI in severe hypophosphataemia with central nervous system involvement.

    We report a 38-year-old woman with extreme hypophosphataemia in whom CT and MRI disclosed bilateral lesions within the basal ganglia, thalamus and occipital lobes. After adequate substitution of phosphate the lesions grossly resolved and the patient recovered. This case is the first to demonstrate that profound changes of serum phosphate may be associated with reversible brain lesions.
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3/92. Attentional grasp in far extrapersonal space after thalamic infarction.

    Studies of animals and humans with focal brain damage suggest that attention in near and far extrapersonal space may be mediated by anatomically separate systems. Thalamic lesions have been associated with spatial neglect, but whether asymmetric attention specific to near or far space occur after thalamic damage has not been explored. It is also unclear if thalamic injury can induce contralesional defective response inhibition.We tested a woman with a left thalamic infarction who reported that, when driving, she had a tendency to veer towards people or objects on the right side of the road. Our patient and four controls performed a line bisection task with a laser pointer in near and far extrapersonal space. The experimenter marked each bisection either from the right of the presented line (right-distractor, RD) or the left (left-distractor, LD). RD and LD trials were pseudo-randomized.Our patient performed similarly to controls (mean -0.7 mm, controls -0.6 mm) on the line bisection task in near space. In far space she erred significantly rightward compared to her performance in near space (p<0.001). Controls performed similarly in near and far space. The experimenter position did not affect our patient's performance on near line bisections, nor did controls demonstrate a distractor effect for the near condition. In the far condition, however, our patient showed a significant distractor effect (LD -3.3 mm, RD 35.3 mm, p<0.001). Controls also demonstrated a distractor effect in the far condition (LD -6.4 mm, RD 0.7 mm, p<0.01), though of much smaller magnitude. Our results suggest that frontal-thalamic systems regulating visual attention may be disrupted by thalamic infarction. Such damage may produce an attentional grasp specific to far extrapersonal space.
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4/92. Thalamic degeneration with negative prion protein immunostaining.

    A 34-year-old woman presented with an insidious 5-year history of cognitive decline and apathy, associated with hypersomnia, ataxia, and dysarthria. magnetic resonance imaging of the brain showed cortical and subcortical atrophy. At autopsy we found abnormalities in the subcortical grey matter and brainstem, with a relatively preserved cerebral cortex. The thalami showed symmetrical neuronal loss and astrocytosis, particularly severe in the dorsal medial nucleus, followed by the lateral nuclei group. Prion protein immunostaining was negative, and there was no spongiform change. No mutations were detected in the prion protein gene.
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5/92. Selective loss of vergence control secondary to bilateral paramedian thalamic infarction.

    The supranuclear pathways for vergence eye movements are poorly understood. The authors report a 57-year-old patient who presented with selective loss of vergence control and dissociation of light and near reaction. MRI showed a symmetric paramedian thalamic infarction without midbrain lesion. The findings suggest that this syndrome is due to an interruption of supranuclear fibers to midbrain vergence neurons.
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6/92. Bilateral basal ganglia-thalamic lesions subsequent to prolonged fetal bradycardia.

    We report two infants with bilateral basal ganglia-thalamic lesions subsequent to prolonged fetal bradycardia. Cardiotocogram revealed severe bradycardia lasting for more than 20 min in both. They demonstrated a significant encephalopathy, abnormal muscle tones and signs of brainstem injury. Clinical or electrical seizures were not observed in either of them. CT during early neonatal period demonstrated decreased tissue attenuation in basal ganglia and thalami in the absence of extensive cortical changes. Both of them developed severe mental retardation and quadriplegia. MRI in late infancy demonstrated abnormal high intensity areas in bilateral basal ganglia, thalami and around central sulci on T2-weighted image. Close correlation between prolonged fetal bradycardia and basal ganglia-thalamic lesion was suggested.
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7/92. Japanese encephalitis with movement disorder and atypical magnetic resonance imaging.

    With the advent of magnetic resonance imaging, brain lesions associated with Japanese encephalitis are increasingly being recognized and correlated with movement disorder. Bilateral haemorrhagic thalamic infarcts on MRI, suggested as a characteristic finding in Japanese encephalitis were conspicuous by their absence in this case report of Japanese encephalitis.
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8/92. Primary neonatal thalamic haemorrhage and epilepsy with continuous spike-wave during sleep: a longitudinal follow-up of a possible significant relation.

    epilepsy with continuous spike-waves during sleep was diagnosed in a child who suffered primary neonatal thalamic haemorrhage, and who was followed from birth to 17 years of age. Early cognitive development was normal. Acquired behavioural problems and cognitive stagnation could be directly related to the epilepsy and not to the initial lesion and posthaemorrhagic hydrocephalus. This case and long-term follow-up data on a few children who suffered primary neonatal thalamic haemorrhage suggest that epilepsy with continuous spike-waves during sleep can be a sequel. Disturbances of thalamocortical interactions could play a role in the still poorly understood syndrome of epilepsy with continuous spike-waves during sleep.
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9/92. Right medial thalamic lesion causes isolated retrograde amnesia.

    Pervasive retrograde amnesia without anterograde memory impairment has rarely been described as a consequence of circumscribed brain damage. We report this phenomenon in a 33 yr-old, right-handed man (JG) in association with the extension in the right thalamus of a previously small, bilateral thalamic lesion. JG presented with a dense amnesia for autobiographical material more than a few years old, with some sparing of recent memories. Furthermore, he was completely unable to recognise famous people or world events. Many other aspects of semantic knowledge were intact and there was no evidence of general intellectual impairment, executive dysfunction or loss of visual imagery. magnetic resonance imaging revealed an acute lesion in the right thalamus and two small, symmetrical, bilateral non-acute thalamic lesions. Follow-up neuropsychological assessment indicated a stable pattern of impaired retrograde and spared anterograde memory over 18 months and psychiatric assessments yielded no evidence of confabulation, malingering or other symptoms to suggest psychogenic amnesia. JG's profile indicates that the division of declarative memory into just two categories - episodic and semantic - is inadequate. Rather, his case adds to the growing body evidence to suggest that world knowledge pertaining to people and events is stored or accessed similarly to autobiographical information and differently from other types of more general factual knowledge. We hypothesize that the right mediodorsal thalamic nucleus and immediately surrounding regions comprise the central processing mechanism referred to by McClelland (Revue Neurologique, 150 (1994) 570) and Markowitsch (Brain research review, 21 (1995) 117) as responsible for inducing and co-ordinating the recall of these sorts of cortically stored memory engrams.
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10/92. Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity.

    Subacute diencephalic angioencephalopathy (SDAE) is a rare and fatal disease of unknown etiology that involves the thalami bilaterally. To date, there have been four cases reported, in which the diagnosis was established only after post mortem examination of the brain. We report two male patients, ages 69 and 41 years, who presented with progressive dementia and somnolence. Radiological evaluation revealed enhancing lesions involving both thalami. The differential diagnosis included a number of neoplastic, inflammatory and vascular processes. In both cases, pathological evaluation of biopsy specimens suggested the diagnosis of SDAE. Despite supportive care, the disease progressed rapidly and both patients died within weeks after initial presentation. The diagnosis was confirmed at autopsy in both cases. SDAE is a rare cause of bithalamic disease that can be mistaken for a neoplasm as well as a number of conditions that necessitate different treatment choices. The histopathological findings can establish the diagnosis when combined with radiological and clinical information. This report emphasizes the utility of stereotactic biopsy in early diagnosis of SDAE.
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