Hashimoto's encephalopathy presenting as pseudobulbar palsy.

INTRODUCTION: Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP). CASE PRESENTATION: A 14-year-old male was admitted with right-sided weakness, dysphagia, speech disorder, and aggressiveness. Brain magnetic resonance imaging showed increased intensity in bilateral temporal, insular cortex, amygdala, and parahippocampal area on T2-weighted and fluid-attenuated inversion recovery images. Autoimmune encephalitis was considered as the patient had subacute onset of psychiatric and motor disturbances with normal findings for cerebrospinal fluid. N-methyl-D-aspartate receptor, anti-glutamate-type α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 and 2, anti-contactin-associated protein-like 2, anti-gamma-aminobutyric acid receptor, anti-Leucine-rich, and glioma-inactivated 1 antibodies were negative but the anti-thyroperoxidase (antiTPO) level was greater than 998 IU/ML (n:0-9). Steroid therapy was initiated as pulse therapy and maintained with 2-mg/kg/day dose with the diagnosis of HE. He was symptom free for 6 months. In the follow-up period, he had two recurrences which responded to steroid therapy. CONCLUSION: The common causes of PSP are demyelinating, vascular, and motor neuron diseases and congenital malformations of the opercular or insular cortex. However, there are no cases of PSP developing after any autoimmune encephalitis. This case highlights the importance of early detection of antiTPO antibodies with the findings of PSP due to autoimmune encephalitis.

Pseudobulbar dysarthria in the initial stage of motor neuron disease with dementia: a clinicopathological report of two autopsied cases.

We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism.

Treatment of pseudobulbar affect with citalopram in a patient with progressive multifocal leukoencephalopthy.

Pseudobulbar affect (PBA) manifests in a variety of neurologic illnesses suggesting a heterogeneous pathophysiology with common underpinnings. We report successful treatment of PBA with a selective serotonin reuptake inhibitor (SSRI) in a 54-year-old woman following progressive multifocal leukoencephalopathy (PML). In light of recent focus on dextromethorphan/quinidine (DM/Q) for the treatment of PBA, the clinician is reminded of the effectiveness of SSRIs.

Síndrome biopercular producido por lesión unilateral / Biopercular syndrome caused by unilateral ischemia: report of one case

Biopercular syndrome is a labio-facio-pharyngeal-laryngeal-gloso-masticatory diplegia, with automatic dissociation of movements. Ischemia is the most common etiology when it occurs bilaterally in the opercular area, but it has been also described in patients with bilateral subcortical lesions. There arefew cases described with unilateral lesions. We report a 76-year-old woman who developed a biopercular syndrome caused by unilateral ischemic lesion ofthe right middle cerebral artery confirmed by magnetic resonance imaging and cerebral SPECT.

Perisylvian polymicrogyria, infantile spasms and arthrogryposis: the severe end of the spectrum of congenital bilateral perisylvian polymicrogyria.

Congenital bilateral perisylvian polymicrogyria (CBPP) is the most frequent type of polymicrogyria in children. A 3-month-old male patient is described here with the combination of CBPP, infantile spasms and arthrogryposis. Only four patients have been reported earlier in the literature with this combination. Three of them had epilepsy. These patients represent the more severe phenotype of CBPP, characterized by early onset of symptoms, epilepsy, mental retardation, pseudobulbar palsy and arthrogryposis.

Congenital bilateral perisylvian syndrome with partial epilepsy. Case report with long-term follow-up.

Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder characterised by pseudobulbar palsy, cognitive deficits and epilepsy associated with bilateral perisylvian cortical dysplasia on neuroimaging studies. We report a long-term follow-up of a 18-years girl diagnosed with CBPS according to the typical clinical and magnetic resonance imaging (MRI) features. The patient showed faciopharyngoglossomasticatory diplegia, severe dysarthria, ataxia, spastic quadriparesis and severe mental retardation. Brain MRI evidenced bilateral perisylvian cortical dysplasia. Since early life she suffered from complex febrile seizures and epilepsy consisting of complex partial attacks with affective manifestations associated with centro-temporal EEG abnormalities. During 18 years of follow-up she was treated with phenobarbital, carbamazepine, lamotrigine, gabapentin but did not show any significant clinical improvement. Subsequently, monotherapy with phenytoin (PHT) was followed by a significant clinical improvement. At age 17, because of adverse effects, PHT was gradually substituted by topiramate (TPM). Full control of seizures was obtained at the age of 17 years with TPM. EEG abnormalities throughout the years have been reduced according to the clinical course. These findings emphasised the importance of long-term follow-up, suggesting that the prognosis for epilepsy may not be predicted based on the early response to treatment or on the presence of structural encephalic abnormalities, as reported in the literature.

[Early tracheostomy in patients with tumors in the posterior cranial fossa during postoperative period]. / Ranniaia trkheostomiia u bol'nykh s opukholiami zadnei cherepnoi iamki v posleoperatsionnom periode.

Analyzed in the paper is the early postoperative management of 50 neurosurgery patients operated on the structures of the posterior cranial fossa, primarily, in as far as the respiratory problems and their solution by early tracheostomy are concerned. A classification of indications for early tracheostomy as well as an algorithm of the management of such patients are described.

Treatment of pseudobulbar laughter after gamma knife thalamotomy.

We describe a case of pathological laughter after gamma knife thalamotomy which resolved after treatment with sertraline. It is important to identify this potentially treatable complication of surgical therapy.

Doença de Alzheimer: manifestaçöes neurológicas / Alzheimer's disease: neurologic manifestations

Os sinais neurológicos na doença de Alzheimer (DA) nä têm sido considerados com o mesmo interesse que as manifestaçöes cognitivas, os de comportamento e psicológicas e os funcionais. A presente revisäo contempla as principais manifestaçöes neurológicas passíveis de ocorrer no curso da DA, divididas em quatro tópicos: sinais extrapiramidas e discinesias (parkinsonismo, discinesia oromandibular, mioclonia), sinais piramidais, paralisia pseudobulbar), disfunçäo integradativa (distúrbios de postura, transtornos de marcha, sinais cerebelares, paraplegia em flexäo de origem cerebral) crises convulsivas. Baseados em ampla revisäo bibliográfica, säo apresentados dados numéricos representativos da pervalência dos sinais neurológicos mais importantes de manifestaçäo no curso da doença e comparados com sua presença em condiçöes normais. Säo ainda feitas consideraçöes de correlaçäo dos sinais neurológicos a diversas características evolutivas e da sua importância em relaçäo ao prognóstico da doença