1/166. Serial electroencephalographic findings in patients with MELAS.To clarify the electroencephalographic characteristics of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS), the medical records and electroencephalograms of six patients with MELAS and two of their relatives with MELA (mitochondrial myopathy, encephalopathy, and lactic acidosis, without strokelike episodes) were retrospectively reviewed. All have a point mutation in the mitochondrial dna at nucleotide position 3243. The electroencephalograms (n = 79) were divided into four groups according to the time relation to the strokelike episode: (1) before the first strokelike episode, (2) within 5 days after the strokelike episode (acute stage), (3) between 6 days and 1 month after the strokelike episode (subacute stage), and (4) more than 1 month after the strokelike episode (chronic stage). In the acute stage, 10 of the 11 electroencephalograms (9 strokelike episodes in four patients) revealed focal high-voltage delta waves with polyspikes (FHDPS), which were recognized as ictal electroencephalogram. Ictal events during FHDPS included focal clonic or myoclonic seizure and migrainous headache. In the subacute and chronic stages, focal spikes or sharp waves and 14- and 6-Hz positive bursts were frequently recorded. The authors' results suggest that FHDPSs present a reliable and accurate indicator of a strokelike episode in patients with MELAS.- - - - - - - - - - ranking = 1keywords = encephalopathy (Clic here for more details about this article) |
2/166. Juvenile form of dihydropteridine reductase deficiency in 2 Tunisian patients.Two brothers are described who had juvenile-onset DHPR deficiency. Both were considered normal until six years of age when they developed a fluctuating and progressive encephalopathy combining mental retardation, epilepsy, pyramidal, cerebellar and extrapyramidal signs.- - - - - - - - - - ranking = 0.5keywords = encephalopathy (Clic here for more details about this article) |
3/166. Infantile and juvenile presentations of Alexander's disease: a report of two cases.We describe 2 new cases of Alexander's disease, the first to be reported in belgium. The first patient, a 4-year-old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy: post-mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis. She was the third of heterozygotic twins, the 2 others having developed normally and being alive at age 5 years. The second patient developed at age 10 years and over a decade spastic paraparesis, palatal myoclonus, nystagmus, thoracic hyperkyphosis and thoraco-lumbar scoliosis with radiological findings of bilateral anterior leukoencephalopathy. Brain stereotactic biopsy at age 16 years demonstrated numerous RFs. With these 2 cases, we review the literature on the various clinico-pathological conditions reported as Alexander's disease. We discuss the nosology of this entity and the pathogeny of RFs formation and dysmyelination. Clues to the diagnosis of this encephalopathy in the living patient are briefly described.- - - - - - - - - - ranking = 1keywords = encephalopathy (Clic here for more details about this article) |
4/166. posterior leukoencephalopathy syndrome may not be reversible.The association of an acute reversible encephalopathy with transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinical presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants. The authors discuss a two-year-old male with down syndrome who presented 2 months after allogeneic bone marrow transplantation with severe oculogyric crisis, without other complaints. The patient was being treated for hypertension and was receiving cyclosporine for prophylaxis of graft-vs-host disease. A computed tomography scan of the head revealed marked bilateral lucencies mainly involving the white matter of the occipital lobes, with a few foci of punctate hemorrhage. The condition improved when cyclosporine was discontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors' knowledge, oculogyric crisis as a presentation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients receiving cyclosporine are often receiving other medications that can potentially cause dystonic eye movements, possibly leading to a delay in diagnosis and treatment, which can result in an irreversible neurologic deficit.- - - - - - - - - - ranking = 3.5keywords = encephalopathy (Clic here for more details about this article) |
5/166. A distinct difference in clinical expression of two siblings with Aicardi-Goutieres syndrome.Two sibs with an encephalopathy, including intracerebral calcification and a white matter disease, are reported. In the younger sister, the cerebrospinal fluid showed chronic pleocytosis and clinically she strictly fits to the diagnosis of Aicardi-Goutieres syndrome. Both sisters were affected by a spastic tetraplegia, truncal hypotonia and dystonic posturing, but the clinical course and the neuroradiological findings were milder in the older sister and she showed no cerebrospinal fluid pleocytosis. The present cases and recent reports of intrafamilial variability of Aicardi-Goutieres syndrome may raise interesting aspects as to the limits and criteria of this syndrome.- - - - - - - - - - ranking = 0.5keywords = encephalopathy (Clic here for more details about this article) |
6/166. 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 = 0.30355075876635keywords = injury (Clic here for more details about this article) |
7/166. Spontaneous disappearance of temporo-frontal arachnoid cyst in a child.We report a child with a large temporo-frontal arachnoid cyst which resolved spontaneously. There was no history of a head injury. The patient was a boy aged 1.6 years. Though a large head was pointed out ( 2SD), no therapeutic intervention was made because the relationship of the head circumference and the cyst was not established. No change in cyst size was visualized on the follow-up CTs at the age of 2.5 years and 6 years. At the age of 7 years, the arachnoid cyst had completely disappeared on CT. In order not to overlook a minute change in cyst size, the volumetry of the cyst and the intracranial cavity was performed, using the Photoshop, Macintosh. Both the cyst volume and the volume ratio of the cyst to the intracranial cavity slightly decreased and then increased. It is speculated that the cyst spontaneously ruptured by factors such as extreme breath holding and crying on the presence of the higher intracystic tension which might become a factor to accelerate spontaneous rupture of the cyst. Since a number of paediatric cases of symptomatic arachnoid cysts in need of surgical intervention has been larger than that of adult cases, we can speculate that a large arachnoid cyst might spontaneously resolve more frequently than we had expected. This case demonstrates that the surgical treatment of asymptomatic arachnoid cyst in the middle cranial fossa is not necessarily indicated in children.- - - - - - - - - - ranking = 0.025295896563862keywords = injury (Clic here for more details about this article) |
8/166. Laminar and arch fractures with dural tear and nerve root entrapment in patients operated upon for thoracic and lumbar spine injuries.OBJECTIVE: To determine the neurological outcome in patients with laminar fractures associated with dural tears and nerve root entrapment, operated upon for thoracic and lumbar spine injuries. PATIENT population: Out of 103 patients operated upon consecutively for thoracic and lumbar spine injuries during the period 1990 to 1994 inclusive, 24 (23.3%) patients had laminar fractures out of whom 3 (2.9%) had an associated dural tear and an other 17 (16.5% or 70.8% of the total patients with laminar fractures) had an associated dural tear and nerve root entrapment. RESULTS: Twelve (70.5%) patients had injury at the thoraculumbar junction, 13 (76.5%) had Magerl's type A3 or above, 10 (58.8%) had a kyphotic angle deformity greater than 5 degrees. Seven (41.1%) had their spinal canal's sagittal diameter reduced by at least 50% and two had dislocations. Nine (52.9%) had initial neurological deficits. Four (50%) out of 8 patients with no initial neurological deficits (Frankel E) worsened to Frankel D. However, one patient among the 3 with initial Frankel A improved to Frankel C while both patients with initial Frankel C usefully improved to final Frankel grades D and E respectively. Two of the four patients with initial Frankel D improved to Frankel E, the other 2 remaining unchanged. All in all five patients neurological status improved, 4 worsened and 8 remained unchanged after neurosurgical treatment. CONCLUSIONS: Vertical laminar fractures with dural tears and nerve root entrapment represent a special group of thoracic and lumbar spine injuries that carry a poor prognosis. However, special operative precautions lead to significant improvement in some of them although a majority remain unchanged or even worsened.- - - - - - - - - - ranking = 0.025295896563862keywords = injury (Clic here for more details about this article) |
9/166. Delayed diffuse upper motor neuron syndrome after compressive thoracic myelopathy.A 54-year-old man developed progressive spastic paraparesis beginning 2 weeks after a back injury caused by a subacute compressive thoracic myelopathy attributable to a post-traumatic arachnoid cyst. Three to 18 months after surgical decompression of the thoracic arachnoid cyst, the patient developed a diffuse predominantly upper motor neuron syndrome characterized by spastic quadriparesis, pseudobulbar paresis, and pseudobulbar affect. Retrograde corticospinal tract degeneration and upper motor neuron death after spinal cord injury is recognized. This case suggests that focal upper motor neuron injury can occasionally precipitate diffuse upper motor neuron dysfunction.- - - - - - - - - - ranking = 0.089765498935997keywords = injury, post-traumatic (Clic here for more details about this article) |
10/166. Acute relapsing encephalopathy mimicking acute necrotizing encephalopathy in a 4-year-old boy.A 4-year-old boy showed two episodes of encephalitis/encephalopathy involving disturbed consciousness, convulsion, and paresis associated with the elevated levels of protein and myelin basic protein of the cerebrospinal fluid. MRI studies of the brain revealed symmetrical lesions in the brain stem and thalami at the first episode, and additional lesions were found in the cerebellum involving both the gray and white matter in the second episode. The intensities of MRI lesions were low in T I and high in T2. These episodes were followed by an elevation of the anti-viral antibody titers, for influenza a virus during the first episode and for adenovirus during the second. In the second episode, intravenous methylprednisolone therapy resulted in rapid improvement of his neurological signs.- - - - - - - - - - ranking = 4.5keywords = encephalopathy (Clic here for more details about this article) |
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