Ultrasonographic findings post laryngoplasty in the horse.

Laryngoplasty is commonly used to treat laryngeal hemiplegia in Thoroughbred racehorses. Evaluation of the success of the laryngoplasty is traditionally determined using endoscopy. Laryngeal ultrasonography and normal ultrasonographic appearance have been reported in the standing horse, but post-laryngoplasty and ventriculectomy ultrasonographic evaluation has limited literature coverage. A prospective case series of 10 Thoroughbred racehorses with left laryngeal hemiplegia was examined ultrasonographically and endoscopically prior to 3-10 days, 30-50 days, and 6-12 months after laryngoplasty and ventriculectomy. Anatomical structures and Plica vocalis movements were described and measurements and gradings analyzed by repeated means analysis of variance (P < .05). Postsurgical ultrasonographic visualization of Ventriculus laryngis entrances was possible. The distance between Plica vocalis in exhalation was significantly larger than that during inhalation (P < .05). Pre- and postsurgical caudal Basihyoideum and rostral Cartilago thyroidea depth was significantly different in some instances (P < .05). No significant differences in the Muscularis cricoarytenoideus lateralis measurements were found. Complications in the extra-luminal structures were found in seven horses including soft tissue swelling, seroma, and hematoma. A luminal Plica vocalis abscess and Plica vocalis granuloma were also detected ultrasonographically. Ultrasonography can be used to evaluate the post-laryngoplasty horse for assessing the success of the procedure, monitoring healing, and detecting complications.

A 41-year-old female with progressive multifocal leukoencephalopathy after liver transplant.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by JC virus reactivation. Its occurrence is very rare after solid organ transplantation, especially liver transplantation. We report a patient who received liver transplantation due to liver failure resulting from autoimmune hepatitis and advanced PML presenting with aphasia. A 41-year-old female with a history of liver transplantation who received a usual immunosuppression regimen was admitted with a stroke attack resulting in right hemiplegia 2 months after liver transplantation. Surprisingly, she gradually developed dysarthria and left central facial paresis. A brain MRI showed an abnormal multifocal area with a high T2/flair signal in the deep subcortical white matter of the left hemisphere as well as the splenium of the corpus callosum. PCR evaluation of CSF for JCV was positive while other PCR results were negative. A liver transplant recipient receiving immunosuppressive treatment for a long time could develop PML due to JCV reactivation. Only eight cases of JCV infection were reported after liver transplantation by the time of reporting this case. Unfortunately, there is no definite treatment for PML.

Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis: A 99mTc-HMPAO Brain SPECT Study.

Genetic mutations of sporadic hemiplegic migraine (SHM) are mostly unknown. SHM pathophysiology relies on cortical spreading depression (CSD), which might be responsible for ischemic brain infarction. Cystic fibrosis (CF) is caused by a monogenic mutation of the chlorine transmembrane conductance regulator (CFTR), possibly altering brain excitability. We describe the case of a patient with CF, who had a migrainous stroke during an SHM attack. A 32-year-old Caucasian male was diagnosed with CF, with heterozygotic delta F508/unknown CFTR mutation. The patient experiences bouts of coughing sometimes triggering SHM attacks with visual phosphenes, aphasia, right-sided paresthesia, and hemiparesis. He had a 48-hour hemiparesis triggered by a bout of coughing with hemoptysis, loss of consciousness, and severe hypoxia-hypercapnia. MRI demonstrated transient diffusion hyperintensity in the left frontal-parietal-occipital regions resulting in a permanent infarction in the primary motor area. Later, a brain perfusion SPECT showed persistent diffuse hypoperfusion in the territories involved in diffusion-weighted imaging alteration. Migrainous infarction, depending on the co-occurrence of 2 strictly related phenomena, CSD and hypoxia, appears to be the most plausible explanation. Brain SPECT hypoperfusion suggests a more extensive permanent neuronal loss in territories affected by aura. CF may be then a risk factor for hemiplegic migraine and stroke since bouts of coughing can facilitate brain hypoxia, triggering auras.

Long-term follow up of an adult with alternating hemiplegia of childhood and a p.Gly755Ser mutation in the ATP1A3 gene.

Alternating hemiplegia of childhood (AHC) is a rare neurological disease mainly caused by mutations in the ATP1A3 gene and showing varied clinical severity according to genotype. Patients with a p.Gly755Ser (p.G755S) mutation, one of minor genotypes for AHC, were recently described as having a mild phenotype, although their long-term outcomes are still unclear due to the lack of long-term follow up. Here, we demonstrate the full clinical course of a 43-year-old female AHC patient with p.G755S mutation. Although her motor dysfunction had been relatively mild into her 30 s, she showed a subsequent severe aggravation of symptoms that left her bedridden, concomitant with a recent recurrence of seizure status. The seizures were refractory to anti-epileptic drugs, but administration of flunarizine improved seizures and the paralysis. Our case suggests that the phenotype of AHC with p.G755S mutation is not necessarily mild, despite such a presentation during the patient's younger years.

An Infant With Epilepsy and Recurrent Hemiplegia due to Compound Heterozygous Variants in ATP1A2.

BACKGROUND: Pathogenic heterozygous variants in the ATP1A2 gene have most commonly been associated with familial hemiplegic migraine. However, a wide spectrum of phenotypes that include alternating hemiplegia of childhood and epilepsy have been described. PATIENT DESCRIPTION: We describe a boy who presented at age three months with a complex phenotype that included epilepsy, nonepileptic paroxysmal events, and recurrent hemiplegia. Magnetic resonance imaging demonstrated unilateral cortical edema during a severe episode of hemiplegia that was followed by a persistent mild hemiparesis. RESULTS: Whole-exome sequencing identified a previously reported ATP1A2 missense variant (p.Arg548Cys) classified as pathogenic and a novel missense variant (p.Arg1008Trp) classified as a variant of uncertain significance. After this genetic diagnosis, treatment with flunarizine was initiated and no further episodes of hemiplegia have occurred. CONCLUSIONS: This is only the second report of compound heterozygosity of the ATP1A2 gene. It demonstrates the spectrum of paroxysmal neurological events that can arise as a result of ATP1A2 variants, with unique features overlapping alternating hemiplegia of childhood, hemiplegic migraine, and epilepsy. This child illustrates the diagnostic challenges that these disorders can present and the importance of genetic diagnosis in guiding management.

Atrial myxoma: a rare cause of hemiplegia in children.

BACKGROUND: Atrial myxoma is an uncommon cause of hemiplegia in children. However hemiplegia is the commonest manifestation of atrial myxoma in the paediatric age group. CASE REPORT: An 11-year-old girl presented with left hemiplegia and palpitations. Three months later she had a deepvein thrombosis of the right common iliac vein. MRI of the brain showed a subacute right thalamic infarct, and an ECG showed left atrial and left ventricular hypertrophy. Transthoracic echocardiography revealed a left atrial myxoma impinging on the mitral valve. A diagnosis of left atrial myxoma with multiple thromboembolic events was made. She was placed on anticoagulants until she died while awaiting surgical tumour resection. CONCLUSION: Echocardiography should be done early in children presenting with ischaemic thromboembolic diseases in order to reduce morbidity and mortality rates resulting from cardiac pathology.

Hemiplegia Following Mild Head Injury in a Child with Sturge-Weber Syndrome - A Diagnostic Dilemma.

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder with skin, eye, and brain involvement. Hemiplegia in children with SWS after a mild head injury is known to occur in up to one-fifth of cases. A3-year male child presented with a sudden onset hemiplegia following a mild head injury. He was known to have seizure disorder and was being treated with sodium valproate. CTscan of the brain showed contusion. He was admitted for neurological observations and the patient made complete recovery with conservative treatment. MRI scan of the brain done 5 days later which showed venous malformation of choroid plexus on the left side. These changes were considered to be consistent with a preexisting cerebral lesion which coincidentally got detected at neuroimaging done after the mild head injury. There is need for good seizure control as it is likely to be associated with better neurological outcome. The case emphasizes the need for clinical correlation with findings at neuroimaging in children with SWS presenting with head injuries.

Survival following decompressive hemicraniectomy for hemiconvulsion-hemiplegia-epilepsy syndrome: case report.

Hemiconvulsion-hemiplegia-epilepsy (HHE) is an uncommon epileptic syndrome that affects young children. Typical management includes early initiation of benzodiazepines to abate the initial seizure activity quickly. Patients in whom epilepsy develops require prolonged use of antiepileptic agents. Herniation due to diffuse cerebral edema from HHE is rare; however, decompressive craniectomy has been described as a lifesaving measure. The authors present the case of a patient in whom a decompressive craniectomy was performed. They advocate a proactive approach in the detection and management of cerebral edema in HHE causing intracranial hypertension. In HHE cases that exhibit radiographic evidence of malignant cerebral edema (although not previously described in this disease, but similar to the setting of stroke and trauma), the authors advocate early neurosurgical consultation and evaluation for insertion of an intracranial pressure monitor for those patients who do not have a reliable neurological examination (i.e., Glasgow Coma Scale score ≤ 8).

Hemiconvulsion-hemiplegia-epilepsy syndrome: clinical course and neuroradiological features in a 20-month-old girl.

Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome involves initial sudden and prolonged unilateral convulsive seizures, followed by transient or permanent hemiplegia and epilepsy during infancy or early childhood. Seizures are prolonged, difficult to control and sometimes may require surgery. Hemiplegia varies in intensity, differs from Todd paralysis and disappears in about 20% of cases. Neuroimaging characteristically shows brain atrophy more pronounced on the hemisphere contralateral to the side of hemiplegia with dilation of the ventricular system. A 20-month-old girl presented with left hemiconvulsions and left hemiplegia lasting for a prolonged period. Seizures failed to resolve with various anticonvulsants even after many physician contacts. Characteristic neuroimaging findings, seizure control with carbamazepine and valproate, subsequent recovery of hemiplegia and attainment of developmental milestones observed on follow-up confirmed HHE syndrome. The case highlights the need for good seizure control in this syndrome.

Alternating hemiplegia of childhood with a de novo mutation in ATP1A3 and changes in SLC2A1 responsive to a ketogenic diet.

BACKGROUND: Alternating hemiplegia of childhood (AHC) is a rare condition characterized by an early onset of hemiplegic episodes and other paroxysmal or permanent neurological dysfunctions. Recently, mutations in the ATP1A3 gene have been identified as the causal mechanism of AHC. Regarding the differential diagnosis of AHC, glucose transporter 1 deficiency syndrome may be considered because these two disorders share some paroxystic and nonparoxystic features. PATIENT AND RESULTS: We report a typical case of AHC harboring a de novo mutation in the ATP1A3 gene, together with a duplication and insertion in the SLC2A1 gene who exhibited marked clinical improvement following ketogenic diet. CONCLUSION: Because the contribution of the SLC2A1 mutation to the clinical phenotype cannot be definitely demonstrated, the remarkable clinical response after ketogenic diet led us to the hypothesis that ketogenic diet might be effective in AHC as it provides an alternative energy source for the brain.

Isolated unilateral aplasia of submandibular gland: a rare anomaly detected incidentally on computerized tomography.

Aplasia of the major salivary glands is a very uncommon anomaly, with isolated aplasia of only 1 submandibular gland being even rarer. We report a case of a 60-year-old woman in whom unilateral aplasia of the left submandibular gland was detected incidentally on computerized tomography with no other associated congenital abnormalities.

Predicting acute ischemic stroke by measuring the degree of ocular gaze deviation (Prevost's sign) on CT.

BACKGROUND AND AIM: Ocular gaze deviation (OGD) is a well known clinical observation (Prevost's sign) in patients with acute cerebral ischemic stroke. Although OGD has been observed on CT in acute stroke, no investigation has quantified the degree of OGD in acute stroke. MATERIAL AND METHODS: A blinded prospective comparison was performed of two groups of adult patients who underwent CT of the brain. Group 1 comprised patients with acute hemiplegia or hemiparesis due to middle cerebral artery ischemic stroke. Group 2 included ambulatory outpatients with a history of headache but no clinical neurologic signs or cerebral pathology on CT. The CT images were cropped to only show the orbital contents. A neuroradiologist, who was blinded to the clinical data, then measured the OGD for both groups. The OGD was quantified using the axial planes of the lenses relative to the nasal midline structures, and the bilateral OGD average was calculated. Both groups were also evaluated for conjugate or disconjugate gaze. RESULTS: were analyzed using Fisher's exact test. RESULTS: 10 of 70 patients in group 1 and 15 of 46 patients in group 2 were eligible for analysis. The frequency of conjugate and disconjugate gaze was similar in the two groups (p = 0.596). An averaged OGD of >14° and an OGD >18° in either globe was predictive of the presence of acute stroke (p = 0.0166). CONCLUSION: Measurement of OGD is useful in predicting the presence of acute ischemic stroke.

[Single photon emission computed tomography findings in a case of alternating hemiplegia of childhood in relation to migraine].

Single photon emission computed tomography (SPECT) was performed 3 times during attacks and performed 4 times during postictal periods on a case of alternating hemiplegia of childhood. Hyperperfusion of the corresponding hemisphere to hemiparesis was suggested by asymmetric increase RI uptake during the ictal scans, whereas interictal scans showed symmetric topography of cerebral blood flow. Manifestations except hemiplegia included loss of consciousness and vomiting. These manifestations and ictal SPECT findings are the same evidence as hemiplegic migraine. It suggests that these two disorders have a similar pathophysiology. There are two interesting findings regarding this patient. The first finding is magnetic resonance imaging showed progressive cerebellar atrophy. Second finding is interictal SPECT showed a progressive decrease of cerebral perfusion, especially in cerebellar hemispheres. These two findings suggest alternating hemiplegia of childhood may be a chronic progressive disorder.

Ultrasonographic findings in hemiplegic knees of stroke patients.

Clinical and radiologic asymmetric arthritic differences between paralyzed and nonparalyzed limbs of stroke patients have been reported. Arthritic pathology aggravates motor dysfunction and compromises rehabilitation. Musculoskeletal ultrasonography plays an important role in showing soft tissue and the articular cartilage of the knee. Fifty-nine patients with either ischemic or hemorrhagic stroke-induced right or left hemiplegia were recruited to evaluate soft-tissue and intra-articular cartilage changes in hemiplegic knees of stroke patients using ultrasonography. An additional 15 subjects (30 knees) without knee disease or a history of knee trauma or surgery were used as controls. There were significant differences in suprapatellar effusion and patellar tendinitis between hemiplegic and nonhemiplegic knees. Suprapatellar effusion and pes anserinus tendinitis were correlated with Brunnstrom stage. The length of time since stroke onset was not significantly correlated with positive ultrasonographic findings in hemiplegic knees. In conclusion, ultrasonography is useful for detecting periarticular soft-tissue changes and intra-articular lesions in hemiplegic knees of stroke patients.

[A rare cause of exophthalmos, hydatid cyst of the orbit]. / Une cause rare d'exophtalmie, le kyste hydatique de l'orbite.

INTRODUCTION: The orbital hydatid cyst is a rare (1 to 2%) localization of the Echinococcus granulosus parasite. OBSERVATIONS: An intra-orbital localization of hydatidosis was revealed in two patients by the occurrence of unilateral exophthalmia. After tumoral resection the diagnosis was confirmed by histological examination. COMMENTS: The principle clinical sign of an orbital hydatid cyst is exophthalmia. Imaging is essential for pre-operative diagnosis. Serology is insufficient and treatment is surgical.

[Haemodialysed, hemiparetic patient operated on for aortic aneurysm followed by successful renal transplantation]. / Vascularis encephalopathiában szenvedó krónikus hemodializált betegen végzett aortaaneurysma-mútétet követó vesetranszplantáció.

The authors present the case of a 58 years old male patient. According to his preceding record in 1991 he suffered speech disturbance and left-side hemiparesis due to multiplex vascular lesions. A fresh cerebral ictus caused a right side hemiparesis mainly in the upper limb in 1993 and sensomotororic aphasia has also been developed. In 1995 the patient was begun acute haemodialysis treatment due to his gravis uremic state, then his dialysis was continued 3 times 4 hours weekly. In 1997 the then 56 years old inveterate right-sided hemiparetic patient, treated with chronic haemodialysis requested to be put on the transplantation waiting list. The first thing that had to be done in case of this high-risk patient was the resection of the abdominal aortic aneurysm (38 mm x 67 mm x 115 mm in size) noticed at the ultrasound examination which was carried out in January 1997. The continuity of the vessel was secured by graft-interposition where the arteria mesenterica was also implanted. Following the successful operation, the patient was qualified for the transplantation list and in the February of 1998 a successful kidney-transplantation was carried out. Following the temporary, post-transplantation difficulties (post-operative 5th day acute rejection well-reacting to 3 steroid-shots; the two re-operations due to partial necrosis in the uretero-ureteralis anastomosis and successfully overcoming the Pseudomonas aeruginosa uroinfection) the patient is currently doing well and has no complaint.