A combination of modified Kuhnt-Szymanowski and Celsus-Hotz techniques for correction of entropion and overlong lower eyelids in dogs (40 eyes).

PURPOSE: To describe a surgical approach to correct entropion and overlong lower eyelids in dogs by combining the Celsus-Hotz with the modified Kuhnt-Szymanowski technique. METHODS: Medical records of patients undergoing the described surgical procedure were reviewed. A semilunar-shaped piece of lower eyelid was excised and combined with an angled incision at the most lateral aspect of the wound. Adjacent to this incision a skin flap was mobilized to expose the subcutaneous tissue in the ventral aspects. A four-sided wedge resection was used to shorten the lid margin in variable positions. Following closure of the eyelid margin wedge resection, a wedge of equal width was removed from the lateral skin flap. Skin and subcutaneous tissues were closed in a routine fashion. RESULTS: All surgeries were performed by an ECVO diplomate or resident. The surgery was performed unilaterally in four and bilaterally in 18 dogs. Most common breeds were English Bulldog (n = 7), Saint Bernard (3), Rottweiler (2) and Cane Corso Italiano (2). Median age was 22 months (range 5-100 months). Median follow-up was 30 days (range 9-987 days). A single surgical procedure was sufficient to correct the entropion in 97.5% (39/40) of eyes. CONCLUSION: The combination technique described is a suitable surgical procedure to simultaneously correct lower lid entropion and excessive eyelid length, with the added benefits of a stepped wound closure and a flexible lid margin wedge positioning.

Outcome of phacoemulsification in 71 cats: A multicenter retrospective study (2006-2017).

PURPOSE: To assess outcome of phacoemulsification in cats. METHODS: Records of 71 cats (82 eyes) from five referral centers were reviewed. Groups were divided by cause of cataract (congenital/juvenile [n = 32], traumatic [n = 33], and secondary to uveitis [n = 6]), and group comparisons were performed for the most common complications: postoperative ocular hypertension (POH), uveitis, corneal ulceration, synechia/dyscoria, and posterior capsular opacity (PCO) in three different time periods: immediately postoperatively, at 1-90 days, and at >90 days. RESULTS: Median follow-up was 198 days (interquartile range 64-518 days). The overall visual success rate of the cats with a 12-month follow-up was 92.6% (25/27 eyes). POH occurred in 35/82 (42.6%) eyes. Immediately postoperatively, uveitis was the most common complication in 28/82 eyes (34.1%) followed by corneal ulceration in 22/82 eyes (26.8%). At 1-90 days, uveitis in 41/81 eyes (50.6%) remained the most common complication, followed by synechia/dyscoria in 21/81 eyes (25.9%), corneal ulceration in 16/81 eyes (19.7%), and PCO in 15/81 eyes (18.5%). At >90 days, PCO in 17/47 eyes (36.1%), followed by synechia/dyscoria in 16/47 eyes (34%), was the most common complications. The number of eyes with synechia/dyscoria in the trauma group was higher (13/33 [39.3%]) than in the congenital/juvenile group (5/31 [16.1%]) at 1-90 days (P = .039). No statistical difference was found for the other group comparisons. Three eyes in total were enucleated owing to endophthalmitis, post-traumatic ocular sarcoma, and secondary glaucoma. CONCLUSION: Uveitis in the short-term and PCO and synechia/dyscoria in the long-term were the most common complications following phacoemulsification in cats.

Visual outcome following phacoemulsification in English Cocker Spaniels with suspected progressive retinal atrophy: A retrospective multicenter study of 54 cases (2002-2017).

OBJECTIVE: To describe the visual outcome following phacoemulsification in English Cocker Spaniels (ECS) affected by cataracts and suspected progressive retinal atrophy (PRA). ANIMALS STUDIED: Fifty-four client-owned dogs. PROCEDURES: A multicenter, retrospective study was performed including ECS with suspected PRA which underwent phacoemulsification. PRA was suspected on ophthalmic examination before and after surgery, and/or after electroretinography (ERG) was performed. Visual outcome was assessed by menace response per eye at seven time periods post-surgery (P1= 25-90 days, P2 = 91-180 days, P3 = 181-364 days, P4 = 365-549 days, P5 = 550-729 days, P6 = 730-1094 days, and P7 ≥ 1095 days). Descriptive statistics were performed. Generalized estimating equations were used to identify predictors associated with vision after surgery. Odds ratio and confidence intervals were reported. Significance was set at P < 0.05. Owners were invited to participate in a questionnaire. RESULTS: Phacoemulsification was performed in 85 eyes. Median age at surgery was 9.09 years (min. 2.17 years, max. 13.49 years). At all re-examinations, up to and including P5, significantly more eyes were visual than before surgery (P ≤ 0.003). Odds for vision were significantly increased for eyes that underwent surgery. Electroretinograms were performed in 75/85 eyes that underwent surgery, demonstrating low b-wave amplitudes. There was no significant effect of the age, gender, vision before surgery, presence of dazzle reflex, cataract stage or abnormality on gonioscopy on visual outcome. The questionnaire response rate was 48.2%. Most participants (92.5%) felt that cataract surgery led to improvement of the dog's quality of life. CONCLUSIONS: English Cocker Spaniels with suspected PRA may benefit from phacoemulsification with vision up to 2 years following surgery.

Phenotype of macular corneal dystrophy in Labrador Retrievers: A multicenter study.

OBJECTIVE: To describe the phenotype of canine macular corneal dystrophy (MCD) including the clinical presentation, multimodal ocular imaging, histopathology, and ultrastructural analysis in ten Labrador Retrievers. PROCEDURE: Multicentered data collection. RESULTS: Labrador Retrievers affected by MCD were presented between the age of 4.5 and 6 years of age with a history of cloudy eyes and/or visual impairment. Findings on ophthalmic examination included a diffuse haze of the corneal stroma and multiple, well-demarcated, off-white to yellow-brown, punctate corneal opacities heterogeneous in size. Corneal vascularization developed in most dogs as the disease progressed. Disease progression was associated with increased density of the corneal haze as well as increased number and size of the focal opacities and dogs developed significant visual impairment. Spectral domain-optical coherence tomography revealed multifocal hyper-reflective regions within the stroma. In vivo confocal microscopy revealed marked alterations in reflectivity throughout the entire stroma. Normal keratocytes could not be identified in affected areas. Histopathology showed stromal collagen fibers separated by acidophilic granular material on hematoxylin and eosin stain. The material stained with periodic acid-Schiff and colloidal iron stain but not with Masson trichrome stain, confirming the accumulation of glycosaminoglycans. On electron microscopic ultrastructural examination, keratocytes presented with vacuolated rough endoplasmic reticulum and multiple electron dense cytoplasmic inclusions. In areas keratocytes appeared ruptured, with cell organelles and proteinaceous material grouped together between collagen fibers. CONCLUSION: MCD in Labrador Retrievers has similarities with the human counterpart of the condition and is an important differential diagnosis in dogs with corneal disease.

A Carbohydrate Sulfotransferase-6 (CHST6) gene mutation is associated with Macular Corneal Dystrophy in Labrador Retrievers.

PURPOSE: To locate and identify variants associated with macular corneal dystrophy (MCD) in Labrador Retriever (LR) dogs, in the candidate gene carbohydrate sulfotransferase-6 (CHST6). METHODS: The single coding exon of canine CHST6 was sequenced in one affected LR with MCD and one control LR clinically clear of ocular disease. A further 71 control LR with unknown clinical status were sequenced for the putative causal variant in CHST6. A TaqMan SNP genotyping assay was developed and used to screen an additional 84 dogs (five affected LR and 79 clinically clear LR). Finally, the variant was screened in a third cohort of 89 unrelated LR with unknown clinical status to estimate its allele frequency in the population of LR in the United Kingdom. RESULTS: A single nucleotide polymorphism (SNP) was identified within the coding exon of CHST6, resulting in a missense mutation (c.814C>A, p.R272S). All six LR affected with MCD were homozygous for the mutant allele, while 140/151 control LR were homozygous for the wild-type allele and 11/151 were heterozygous for the mutation, indicating an association with MCD (P < 10-5 ). The mutant allele was present in the unrelated LR cohort at a frequency of 0.017, suggesting carrier and affection rates of 3.3% and 0.028%, respectively. CONCLUSIONS: A missense mutation in the CHST6 gene is strongly associated with autosomal recessive MCD in the LR.

Ocular alkaline injury in four dogs - presentation, treatment, and follow-up - a case series.

PURPOSE: To describe presentation, treatment, and follow-up after unilateral alkaline injuries to the eye in four dogs. MATERIAL AND METHOD: The case notes of four patients that suffered from alkaline injuries to the eye were included in this series. RESULTS: Acute clinical signs included blepharospasm and edema of the eyelids, chemosis and conjunctival hyperemia, conjunctival ischemia, destruction of the corneal epithelium, a whitish haze of the corneal stroma, mild corneal edema, and uveitis. Two patients showed depigmentation of the eyelids. Presumed endothelial cell damage resulted in severe corneal edema in two dogs. Long-term complications included phthisis bulbi, scarring of the eyelids and damage to the meibomian glands, symblepharon formation, conjunctivalization of the cornea, corneal vascularization, pigmentation, and fibrosis. Persisting corneal edema was seen in the dogs with presumed endothelial cell damage. One dog developed a mild bullous keratopathy with superficial corneal ulcerations 4½ years after the injury and had a reduced anterior chamber depth on ultrasound. CONCLUSION: The damage to the ocular structures described here mainly affects the ocular surface. One patient presumably suffered an injury to the ciliary body epithelium resulting in a phthisical globe. Chronic corneal edema, conjunctivalization, and scarring can result in permanent visual impairment. Healing of the ocular surface can take weeks and is associated with a dramatic vascular response. However, a severely vascularized cornea has the potential to clear and allow a good visual outcome long term. Ongoing discomfort was only seen in one case with persistent corneal edema and a secondary bullous keratopathy.

In Vitro Antimicrobial Activity of N-Acetylcysteine against Pathogens Most Commonly Associated with Infectious Keratitis in Dogs and Cats.

To determine the in vitro antimicrobial activity of N-acetylcysteine (NAC) against common pathogens associated with infectious keratitis in dogs and cats, clinical isolates of Staphylococcus (S.) pseudintermedius (n = 20), Streptococcus (St.) canis (n = 10) and Pseudomonas (P.) aeruginosa (n = 7) of canine and feline infectious ulcerative keratitis and a quality control strain (P. aeruginosa DSM 19880) were tested. The minimal inhibitory concentration (MIC) of NAC concentrations was determined using microdilution methodology. For S. pseudintermedius and P. aeruginosa, NAC concentrations in the range of 1.56 mg/mL (0.156%) to 100 mg/mL (10%), and for St. canis, concentrations ranging from 0.195 mg/mL (0.0195%) to 6.25 mg/mL (0.625%) were tested. For S. pseudintermedius, the MIC was 3.12 mg/mL (0.312%) for all tested isolates. For P. aeruginosa isolates and the quality control strain, the MIC ranged from 3.12 mg/mL (0.312%) to 6.25 mg/mL (0.625%). For St. canis, the MIC ranged from 1.56 mg/mL (0.156%) to 3.12 mg/mL (0.312%). NAC has an in vitro antimicrobial activity against three bacterial species commonly found in infectious keratitis in dogs and cats and therefore may be a promising alternative or adjuvant to topical antibiotics. The results warrant a clinical pilot study to assess the potential of NAC to reduce or replace the use of topical antibiotics in line with the One Health approach.

[Macrophthalmos mimics microphthalmos in an 8-month-old Great Swiss Mountain Dog]. / Makrophthalmus imitiert Mikrophthalmus bei einem 8 Monate alten Großen Schweizer Sennenhund.

An 8-month-old Great Swiss Mountain dog was presented with a suspected right-sided microphthalmos, malformed and blind globe which was present since birth. On magnetic resonance imaging an ellipsoid macrophthalmos with absence of the normal retrobulbar tissue was detected. Histology revealed a dysplastic uvea with unilateral cyst formation associated with mild lymphohistiocytic inflammation. The ciliary body covered the posterior side of the lens unilaterally and showed focal metaplastic bone formation. Slight cataract formation as well as diffuse panretinal atrophy and intravitreal retinal detachment was evident. Preoperative diagnostic imaging procedure is recommended in eyes that clinically demonstrate as microphthalmos and are planned to be enucleated. As described in this case report the bulbus may be macrophthalmic which potentially complicates the enucleation. The performance of such a procedure at a site with ophthalmologic and soft tissue expertise is advisable. To the authors' knowledge this is the first report of a macrophthalmos with multiple ocular defects in a dog.

Argon cold atmospheric plasma eradicates pathogens in vitro that are commonly associated with canine bacterial keratitis.

Purpose: To investigate the antimicrobial effect of cold atmospheric plasma (CAP) on pathogens associated with canine bacterial keratitis. Materials and methods: Pseudomonas aeruginosa, Staphylococcus pseudintermedius, and Streptococcus canis strains, which were obtained from dogs with infectious keratitis, were subjected to testing. For each species, four isolates and a reference strain were cultivated on Columbia sheep blood agar and treated with the kiNPen Vet® plasma pen from Neoplas GmbH, Greifswald, Germany. Various continuous treatment durations (0.5, 2, and 5 min) were applied, along with a 0.5-min treatment repeated four times at short intervals. These treatments were conducted at distances of 3 and 18 mm between the agar surface and the pen. Results: CAP treatment reduced bacterial growth in all three species. The most effective treatment duration was 5 min at 3 mm distance, resulting in inhibition zones ranging from 19 to 22 mm for P. aeruginosa, 26-45 mm for S. pseudintermedius and an overall reduction of bacterial growth for Str. canis. Inhibition zones were smaller with decreasing treatment duration and larger distance. Treatment times of 30 s repeated four times and 2 min showed comparable results. Treatment with argon alone did not lead to visible reduction of bacterial growth. Conclusion: Argon cold atmospheric plasma demonstrated a potent in vitro antimicrobial effect on P. aeruginosa, S. pseudintermedius and Str. canis strains with the latter showing the highest sensitivity.

Astrocytes are crucial for survival and maturation of embryonic hippocampal neurons in a neuron-glia cell-insert coculture assay.

Synapses represent specialized cell-cell contact sites between nerve cells. These structures mediate the rapid and efficient transmission of signals between neurons and are surrounded by glial cells. Previous investigations have shown that astrocytes are important for the formation, maintenance, and function of CNS synapses. To study effects of glial-derived molecules on synaptogenesis, we have established an in vitro cell-insert coculture system for E18 rat hippocampal neurons and various glial cell types. Neurons were cultured without direct contact with glial cells for distinct time periods. First, it was confirmed that astrocytes are essential to promote survival of E18 hippocampal neurons. Beginning with 10 days in culture, the concurrent expression of pre- and postsynaptic proteins was observed. Moreover, the colocalization of the presynaptic marker Bassoon and the postsynaptic protein ProSAP1/Shank2 indicated the formation of synapses. A technique was developed that permits the semiautomated quantitative determination of the number of synaptic puncta per neuron. The culture system was used to assess effects of pharmacological treatments on synapse formation by applying blockers and activators of small GTPases. In particular, treatment with lysophosphatidic acid enhanced synaptogenesis in the coculture system.

Ophthalmic and cone derived electrodiagnostic findings in outbred Miniature Long-haired Dachshunds homozygous for a RPGRIP1 mutation.

OBJECTIVE: To investigate ophthalmic and cone-derived electrodiagnostic findings in outbred Miniature Long-haired Dachshunds (MLHD) homozygous for a mutation in the RPGRIP1 gene previously associated with cone-rod dystrophy 1 (cord1). ANIMALS: A total of 36 MLHD homozygous for the RPGRIP1 mutation and 23 dogs clear of the mutation (control group). PROCEDURES: The dogs underwent ophthalmic examination and photopic electroretinogram (ERG) recordings. RESULTS: None of the control dogs presented with clinical or ophthalmic signs consistent with cord1. Amongst the dogs homozygous for the mutation one presented with bilateral symmetrical total retinal atrophy. None of the other dogs in this group showed signs consistent with cord1. Photopic ERG recordings were available in 23 control dogs and 34 dogs homozygous for the mutation. Photopic a- and b-waves following four light stimuli (3 cdS/m(2) ) at a rate of 5.1 Hz were not significantly different between groups. The amplitudes of the 30 Hz flicker (128 flashes, 3 cdS/m(2) ) response were significantly reduced in the dogs homozygous for the PRGRIP1 mutation. The difference in age between the two groups did not significantly affect the difference. CONCLUSION: Homozygosity of the RPGRIP1 mutation does not invariably result in early onset cord1. However, cone derived ERG recordings show evidence of a reduced cone or inner retinal function in homozygous but clinically normal MLHD. Modifying genes that have yet to be identified may influence an individual dog's risk of developing the blinding cord1 and also the age of onset and rate of progression.

Phenotypic variation and genotype-phenotype discordance in canine cone-rod dystrophy with an RPGRIP1 mutation.

PURPOSE: Previously, a 44 bp insertion in exon 2 of retinitis pigmentosa GTPase interacting protein 1 (RPGRIP1) was identified as the cause of cone-rod dystrophy 1 (cord1), a recessive form of progressive retinal atrophy (PRA) in the Miniature Longhaired Dachshund (MLHD), a dog model for Leber congenital amaurosis. The cord1 locus was mapped using MLHDs from an inbred colony with a homogeneous early onset disease phenotype. In this paper, the MLHD pet population was studied to investigate phenotypic variation and genotype-phenotype correlation. Further, the cord1 locus was fine-mapped using PRA cases from the MLHD pet population to narrow the critical region. Other dog breeds were also screened for the RGPRIP1 insertion. METHODS: This study examined phenotypic variation in an MLHD pet population that included 59 sporadic PRA cases and 18 members of an extended family with shared environment and having six PRA cases. Ophthalmologic evaluations included behavioral abnormalities, responses to menace and light, fundoscopy, and electroretinography (ERG). The RPGRIP1 insertion was screened for in all cases and 200 apparently normal control MLHDs and in 510 dogs from 66 other breed. To fine-map the cord1 locus in the MLHD, 74 PRA cases and 86 controls aged 4 years or more were genotyped for 24 polymorphic markers within the previously mapped cord1 critical region of 14.15 Mb. RESULTS: Among sporadic PRA cases from the MLHD pet population, the age of onset varied from 4 months to 15 years old; MLHDs from the extended family also showed variable onset and rate of progression. Screening for the insertion in RPGRIP1 identified substantial genotype-phenotype discordance: 16% of controls were homozygous for the insertion (RPGRIP1(-/-)), while 20% of PRA cases were not homozygous for it. Four other breeds were identified to carry the insertion including English Springer Spaniels and Beagles with insertion homozygotes. The former breed included both controls and PRA cases, yet in the latter breed, cone ERG was undetectable in two dogs with no clinically apparent visual dysfunction. Notably, the insertion in the Beagles was a longer variant of that seen in the other breeds. Fine-mapping of the cord1 locus narrowed the critical region on CFA15 from 14.15 Mb to 1.74 Mb which still contains the RPGRIP1 gene. CONCLUSIONS: Extensive phenotypic variations of onset age and progression rate were observed in PRA cases of the MLHD pet population. The insertion in RPGRIP1 showed the strongest association with the disease, yet additional as well as alternative factors may account for the substantial genotype-phenotype discordance.

Suspected sphenoid bone osteomyelitis causing visual impairment in two dogs and one cat.

OBJECTIVE: To present the clinical and magnetic resonance imaging (MRI) characteristics of sphenoid bone osteomyelitis. PROCEDURES: Two dogs (English Springer Spaniel--ESS, Golden Retriever--GR) and one cat (Domestic Long Haired) were presented with a 2-14-day history of visual deficits and reduced pupillary light reflexes. Investigations included physical, ophthalmologic and neurological examination as well as hematology, serum biochemistry, MRI of the head and cerebrospinal fluid (CSF) analysis. RESULTS: MRI changes included thickening of the sphenoid bone and a loss of normal bone marrow signal on T1W MRI. Enhancement of the sphenoid bones, ventral meninges and ventral surface of the brain was present using paramagnetic contrast medium. CSF analysis was abnormal in the two dogs with increased cellularity, neutrophilic pleocytosis, intracellular bacteria and increased total protein in one, and with lymphocytic pleocytosis in another. CSF analysis was normal in the cat. An underlying cause for the osteomyelitis could not be identified. The use of broad-spectrum antibiotics for 3-6 weeks combined with anti-inflammatory medications proved effective. Full clinical recovery occurred with no relapse during the follow up time of 7 (ESS) and 4 (Domestic Long Haired) years. The GR relapsed 10 months after treatment and recovered following a second 3-week course of broad-spectrum antibiotics with no relapse during the following 3 years. CONCLUSION: Visual pathway deficits in dogs and cats may be due to sphenoid bone osteomyelitis. MRI and CSF analysis can assist diagnosing this potentially treatable condition. To the authors' knowledge this is the first report of sphenoid bone osteomyelitis in these species.

Retrobulbar steatitis and meningitis/empyema secondary to right otitis media, right otitis interna and an inflammatory polyp in a cat.

The purpose was to describe a case of retrobulbar steatitis and meningitis in a cat caused by otitis media, otitis interna and an inflammatory polyp in the middle ear. Investigations included ophthalmic and neurological examinations, haematology and biochemistry, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis and aerobic, anaerobic bacterial and fungal culture of material from the middle ear. A 6-year-old female neutered domestic-short-haired cat presented with right-sided exophthalmos and resistance to retropulsion. Anisocoria, reduced corneal sensation and oculocephalic movements and low Schirmer tear test (STT1) were found. An MRI revealed the right external ear canal and tympanic bulla to be fluid filled with presence of a thickened contrast-enhancing mucosa. Contrast enhancement extended into the calvarium, as marked thickening and contrast enhancement of the pachymeninges, and further rostrally through the right orbital fissure into the orbit. The retrobulbar tissues were swollen, resulting in mild exophthalmos. Enrichment culture of material from the middle ear revealed Klebsiella pneumonia susceptible to marbofloxacin. The cat underwent a total ear canal ablation with removal of the cartilaginous cuff at the external auditory meatus and local debridement and curettage. A polypoid structure was removed from the middle ear. No bulla osteotomy was performed. Horner's syndrome was present immediately following surgery. The cat received a course of systemic dexamethasone (1mg/cat SID/3 weeks) tapered off and marbofloxacin (2mg/kg SID/2 weeks). Follow up at 120 days showed no recurrence of the symptoms. The Horner's syndrome and low STT1 had resolved and returned to normal values respectively. This case highlights the importance of MRI in the investigation of retrobulbar diseases. MRI is a useful tool for the evaluation of the extent of the lesion and the appearance of adjacent structures in which additional potentially life-threatening abnormalities, such as meningitis, can be seen.

Orbital paraganglioma in a dog.

A 10-year-old Rottweiler presented with right-sided moderately painful exophthalmia, blindness, absence of dazzle and pupillary light reflexes, a swollen optic nerve head and ventrolateral indentation of the globe. On magnetic resonance imaging, a 3 x 2 x 2 cm mass with a fluid filled center and contrast-enhancing periphery was noted posteriolateral of the globe. Orbital ultrasound was used for a guided fine needle aspirate of the mass. Cytology revealed moderate numbers of polygonal cells with lightly basophilic cytoplasm. Several cells showed nuclear pseudoinclusions. Histopathology following exenteration of the orbit revealed an infiltrative, extradural neoplasm surrounding the optic nerve. Cells were arranged in packets. Neoplastic cells were immunopositive for neuron specific enolase, synaptophysin and chromogranin A and immunonegative for cytokeratin. Findings were consistent with an extra-adrenal paraganglioma (neuroendocrine tumour). Although complete excision could not be confirmed on histopathology, the owners reported no apparent tumour recurrence 25 months after surgery. In conclusion a paraganglioma should be considered as a differential diagnosis of an orbital mass.

Normal sulfation levels regulate spinal cord neural precursor cell proliferation and differentiation.

BACKGROUND: Sulfated glycosaminoglycan chains are known for their regulatory functions during neural development and regeneration. However, it is still unknown whether the sulfate residues alone influence, for example, neural precursor cell behavior or whether they act in concert with the sugar backbone. Here, we provide evidence that the unique 473HD-epitope, a representative chondroitin sulfate, is expressed by spinal cord neural precursor cells in vivo and in vitro, suggesting a potential function of sulfated glycosaminoglycans for spinal cord development. RESULTS: Thus, we applied the widely used sulfation inhibitor sodium chlorate to analyze the importance of normal sulfation levels for spinal cord neural precursor cell biology in vitro. Addition of sodium chlorate to spinal cord neural precursor cell cultures affected cell cycle progression accompanied by changed extracellular signal-regulated kinase 1 or 2 activation levels. This resulted in a higher percentage of neurons already under proliferative conditions. In contrast, the relative number of glial cells was largely unaffected. Strikingly, both morphological and electrophysiological characterization of neural precursor cell-derived neurons demonstrated an attenuated neuronal maturation in the presence of sodium chlorate, including a disturbed neuronal polarization. CONCLUSIONS: In summary, our data suggest that sulfation is an important regulator of both neural precursor cell proliferation and maturation of the neural precursor cell progeny in the developing mouse spinal cord.

The syndrome of perisylvian polymicrogyria with congenital arthrogryposis.

BACKGROUND: Bilateral perisylvian polymicrogyria (BPP) is a well-recognized malformation of cortical development commonly associated with epilepsy, cognitive impairment, and oromotor apraxia. Reports have suggested the association of BPP with arthrogryposis multiplex congenita. We sought to investigate the clinical, electrophysiological, and neuroradiological features of this combined syndrome to determine if there are unique features that distinguish BPP with arthrogryposis from BPP alone. METHODS: Cases of BPP with congenital arthrogryposis were identified from a large research database of individuals with polymicrogyria. Clinical features (including oromotor function, seizures, and joint contractures), MR brain imaging, and results of neuromuscular testing were reviewed. RESULTS: Ten cases of BPP with congenital arthrogryposis were identified. Most cases had some degree of oromotor apraxia. Only a few had seizures, but a majority of cases were still young children. Electrophysiological studies provided evidence for lower motor neuron or peripheral nervous system involvement. On brain imaging, bilateral polymicrogyria (PMG) centered along the Sylvian fissures was seen, with variable extension frontally or parietally; no other cortical malformations were present. We did not identify obvious neuroimaging features that distinguish this syndrome from that of BPP without arthrogryposis. CONCLUSIONS: The clinical and neuroimaging features of the syndrome of BPP with congenital arthrogryposis appear similar to those seen in cases of isolated BPP without joint contractures, but electrophysiological studies often demonstrate coexistent lower motor neuron or peripheral nervous system pathology. These findings suggest that BPP with arthrogryposis may have a genetic etiology with effects at two levels of the neuraxis.

Corneal squamous cell carcinoma in a Border Collie.

A 6-year-old, female, spayed Border Collie was presented to the Unit of Comparative Ophthalmology at the Animal Health Trust with a 6-month history of a progressive nonpainful opacity of the left cornea. A keratectomy was performed and the tissue submitted for histopathology. The diagnosis was squamous cell carcinoma. There has been no recurrence of the neoplasm to date (5 months). Canine corneal squamous cell carcinoma (SCC) has not been reported previously in the UK.