1/115. Atypical herpes simplex encephalitis presenting as operculum syndrome.This case report demonstrates the course of herpes simplex virus cerebritis in a patient aged 7 years 2 months who presented with non-specific symptoms followed by an epileptic attack. Subcortical, bilateral opercular and bilateral thalamic lesions were detected, but the temporal and inferior frontal lobes were spared. The patient developed anarthria, impairment of mastication and swallowing consistent with operculum syndrome. diagnosis was made by magnetic resonance imaging and elevation of oligoclonal antibodies specific to herpes simplex virus in cerebrospinal fluid after an unexpectedly negative polymerase chain reaction test.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
2/115. dysarthria associated with giant cell arteritis.Over a one month period, a 74-year-old man developed typical features of giant cell arteritis (GCA) including visual changes, headache, scalp tenderness, and an elevated erythrocyte sedimentation rate. In addition, he had reproducible painless dysarthria that was precipitated by chewing or prolonged talking and was relieved by resting the jaw. The dysarthria with chewing along with the other classical symptoms of GCA subsided with treatment. To our knowledge this is the first report of painless dysarthria associated with GCA.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
3/115. Acute dysarthria induced by low dose methotrexate therapy in a patient with erythrodermic cutaneous T-cell lymphoma: an unusual manifestation of neurotoxicity.Neurotoxicity has been associated on rare occasions with methotrexate therapy. We now report the case of a 71-year-old man with erythrodermic cutaneous T-cell lymphoma who developed symptoms of dysarthria and inco-ordination within 1 month of the initiation of oral methotrexate; discontinuation of the therapy then resulted in a gradual resolution of the problems.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
4/115. Progressive dysarthria. case reports and a review of the literature.Two patients presenting with progressive dysarthria as the single initial manifestation of a neurodegenerative condition are described. The nature of the dysarthria as well as the additional symptoms that developed in the course of the disorder are very different in these two cases. Nevertheless, neuroimaging findings are strikingly similar and suggest bilateral involvement of posterior inferior frontal lobe structures, mainly in the dominant cerebral hemisphere. The clinical syndrome of these patients can therefore be considered an example of frontotemporal degeneration presenting without dementia or compartmental alteration, at least in the early stages. This broadens the clinical spectrum of frontotemporal degeneration and demonstrates the need for a syndromal subclassification of this nosological entity.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
5/115. dysarthria during basilar artery balloon occlusion.A 49-year-old woman presenting with recurrent, reversible brainstem symptoms and a distal basilar artery aneurysm underwent balloon test occlusion. Five minutes after balloon inflation she developed a reversible isolated dysarthria. Despite failing the test occlusion (and after an additional brainstem event), the patient underwent surgery with placement of a clip across the basilar artery. The operation was tolerated without complication. The authors conclude that 1) pure dysarthria may be a symptom of temporary basilar artery occlusion and 2) balloon testing may overestimate the risk of basilar artery clipping.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
6/115. Bilateral contemporaneous posteroventral pallidotomy for the treatment of Parkinson's disease: neuropsychological and neurological side effects. Report of four cases and review of the literature.The authors report the underestimated cognitive, mood, and behavioral complications in patients who have undergone bilateral contemporaneous pallidotomy, as seen in their early experience with functional neurosurgery for Parkinson's disease (PD) that is accompanied by severe motor fluctuations before pallidal stimulation. Four patients, not suffering from dementia, with advanced (Hoehn and Yahr Stages III-IV), medically untreatable PD featuring severe "on-off" fluctuations underwent bilateral contemporaneous posteroventral pallidotomy (PVP). All patients were evaluated according to the Core Assessment Program for Intracerebral Transplantations (CAPIT) protocol without positron emission tomography scans but with additional neuropsychological cognitive, mood, and behavior testing. For the first 3 to 6 months postoperatively, all patients showed a mean improvement of motor scores on the Unified Parkinson's Disease Rating Scale (UPDRS), in the best "on" (21%) and worst "off" (40%) UPDRS III motor subscale, a mean 30% improvement in the UPDRS II activities of daily living (ADL) subscore, and 60% on the UPDRS IV complications of treatment subscale. Dyskinesia disappeared almost completely, and the mean daily duration of the off time was reduced by an average of 60%. Despite these good results in the CAPIT scores, one patient experienced a partially regressive corticobulbar syndrome with dysphagia, dysarthria, and increased drooling. No emotional lability was found in this patient, but he did demonstrate severe bilateral postoperative pretarsal blepharospasm (apraxia of eyelid opening), which interfered with walking and which required treatment with high-dose subcutaneous injections of botulinum toxin. No patient showed visual field defects or hemiparesis, but postoperative depression, changes in personality, behavior, and executive functions were seen in two individuals. Postoperative abulia was reported by the family of one patient, who lost his preoperative aggressiveness and drive in terms of ADL, speech, business, family life, and hobbies, and became more sleepy and fatigued. One patient reported postoperative mental automatisms, such as compulsive mental counting, and circular thoughts and reasoning during off phases; postoperative depression was found in two patients. However, none of the patients demonstrated these symptoms during intraoperative microelectrode stimulation. These findings are compatible with previous reports on bilateral pallidal lesions. A progressive lowering of UPDRS subscores was seen after 12 months, consistent with the progression of the disease. Bilateral simultaneous pallidotomy may be followed by emotional, behavioral, and cognitive deficits such as depression, obsessive-compulsive disorders, and loss of psychic autoactivation-abulia, as well as disabling corticobulbar dysfunction and apraxia of eyelid opening, in addition to previously described motor and visual field deficits, which make this surgery undesirable even though significant improvement in motor deficits can be achieved.- - - - - - - - - - ranking = 3keywords = ms (Clic here for more details about this article) |
7/115. Nonconvulsive status epilepticus in a child with congenital bilateral perisylvian syndrome.A 9-year-old male with congenital bilateral perisylvian syndrome is described. He had pseudobulbar palsy, mental retardation, and intractable epilepsy. Computed tomography and magnetic resonance images of the brain demonstrated bilateral perisylvian malformations and a diffuse pachygyric appearance. At 8 years of age, he had episodes of excessive drooling, fluctuating impairment of consciousness, unsteady sitting, and frequent head drop that lasted several days. The electroencephalogram demonstrated continuous diffuse slow spike and waves. These findings suggested atypical absence status epilepticus. Intravenous administration of diazepam resulted in transient improvement of clinical and electroencephalographic findings. status epilepticus recurred within several minutes after diazepam administration. Although no patient has been reported to have a history of status epilepticus among those affected by this syndrome, it seems that atypical absence status can occur more frequently than expected, as seen in Lennox-Gastaut syndrome. After recognition and confirmation of nonconvulsive status epilepticus, immediate treatment must be attempted.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
8/115. Vagal and hypoglossal Bell's palsy.A 7-year-old boy was referred because of a sudden change to nasal speech, dysarthria for words with explosive consonants in speech, and nasal regurgitation of fluids. The symptoms arose over 1 week following a capricious episode of acute asthmatic bronchitis. Physical and neurologic examinations were normal except for a left deviation of the uvula, accompanied by a "curtain" movement of the posterior pharyngeal wall against the opposite side, and a left deviation of the protruded tongue. No vascular, traumatic, infectious, neoplastic, or neurologic causes could be identified. No therapy was administered. Full recovery occurred 4 months later. The diagnosis was idiopathic vagal and right hypoglossal nerve palsy (Bell's palsy).- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
9/115. Woodhouse and Sakati syndrome (MIM 241080): report of a new patient.A 32-year-old male with Woodhouse Sakati syndrome (MIM 241080) is described. Two of the proband's brothers also have diabetes mellitus and similar facial features, however they are not dysarthric. An affected older brother died of an unknown cause at age 30. This confirms autosomal recessive inheritance.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
10/115. A case of spinocerebellar ataxia accompanied by severe involvement of the motor neuron system.We report a sporadic case of spinocerebellar ataxia accompanied by later but severe involvement of the motor neuron system. A 72-year-old man began to show ataxia and dysarthria at age 66 years. Neurological examinations revealed saccadic eye movement, slurred speech, truncal ataxia, pyramidal sign, and urinary disturbance. Neither history of alcoholism nor hereditary factors were found. He developed muscular atrophy of the lower and upper extremities and limb ataxia within three years. Superficial and deep sensations were diminished in both feet four years after onset. Thus, he presented with cerebellar ataxia, bulbar sign, upper and lower motor neuron symptoms, sensory disturbance, and autonomic sign after six years at age 72. The level of serum, creatine phosphokinase (CPK) was increased, and muscle biopsy showed marked neurogenic change. magnetic resonance imaging (MRI) revealed mild cerebellar and pontine atrophy. Although the combination of spinocerebellar ataxia and motor neuron disease is very rare, the present case suggests the inter-relation of the spinocerebellar and motor neuron systems, and presents peripheral neuropathy as a subtype of multisystem atrophy.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
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