The neural underpinnings of reading skill in deaf adults

Publication date: September 2016
Source:Brain and Language, Volume 160
Author(s): Karen Emmorey, Stephen McCullough, Jill Weisberg
We investigated word-level reading circuits in skilled deaf readers (N=14; mean reading age=19.5years) and less skilled deaf readers (N=14; mean reading age=12years) who were all highly proficient users of American Sign Language. During fMRI scanning, participants performed a semantic decision (concrete concept?), a phonological decision (two syllables?), and a false-font control task (string underlined?). No significant group differences were observed with the full participant set. However, an analysis with the 10 most and 10 least skilled readers revealed that for the semantic task (vs. control task), proficient deaf readers exhibited greater activation in left inferior frontal and middle temporal gyri than less proficient readers. No group differences were observed for the phonological task. Whole-brain correlation analyses (all participants) revealed that for the semantic task, reading ability correlated positively with neural activity in the right inferior frontal gyrus and in a region associated with the orthography-semantics interface, located anterior to the visual word form area. Reading ability did not correlate with neural activity during the phonological task. Accuracy on the semantic task correlated positively with neural activity in left anterior temporal lobe (a region linked to conceptual processing), while accuracy on the phonological task correlated positively with neural activity in left posterior inferior frontal gyrus (a region linked to syllabification processes during speech production). Finally, reading comprehension scores correlated positively with vocabulary and print exposure measures, but not with phonological awareness scores.



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Proverb comprehension in individuals with agenesis of the corpus callosum

Publication date: September 2016
Source:Brain and Language, Volume 160
Author(s): Jamie L. Rehmel, Warren S. Brown, Lynn K. Paul
Comprehension of non-literal language involves multiple neural systems likely involving callosal connections. We describe proverb comprehension impairments in individuals with isolated agenesis of the corpus callosum (AgCC) and normal-range general intelligence. Experiment 1 compared Gorham Proverb Test (Gorham, 1956) performance in 19 adults with AgCC and 33 neurotypical control participants of similar age, sex, and intelligence. Experiment 2 used the Proverbs subtest of the Delis-Kaplan Executive Function System (D-KEFS, 2001) to compare 19 adults with AgCC and 17 control participants with similar age, sex, and intelligence. Gorham Proverbs performance was impaired in the AgCC group for both the free-response and multiple-choice tasks. On the D-KEFS proverbs test, the AgCC group performed significantly worse on the free-response task (and all derivative scores) despite normal levels of performance on the multiple-choice task. Covarying verbal intelligence did not alter these outcomes. However, covarying a measure of non-literal language comprehension considerably reduced group differences in proverb comprehension on the Gorham test, but had little effect on the D-KEFS group differences. The difference between groups seemed to be greatest when participants had to generate their own interpretation (free response), or in the multiple choice format when the test included many proverbs that were likely to be less familiar. Taken together, the results of this study clearly show that proverb comprehension is diminished in individuals with AgCC compared to their peers.



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The neural circuits recruited for the production of signs and fingerspelled words

Publication date: September 2016
Source:Brain and Language, Volume 160
Author(s): Karen Emmorey, Sonya Mehta, Stephen McCullough, Thomas J. Grabowski
Signing differs from typical non-linguistic hand actions because movements are not visually guided, finger movements are complex (particularly for fingerspelling), and signs are not produced as holistic gestures. We used positron emission tomography to investigate the neural circuits involved in the production of American Sign Language (ASL). Different types of signs (one-handed (articulated in neutral space), two-handed (neutral space), and one-handed body-anchored signs) were elicited by asking deaf native signers to produce sign translations of English words. Participants also fingerspelled (one-handed) printed English words. For the baseline task, participants indicated whether a word contained a descending letter. Fingerspelling engaged ipsilateral motor cortex and cerebellar cortex in contrast to both one-handed signs and the descender baseline task, which may reflect greater timing demands and complexity of handshape sequences required for fingerspelling. Greater activation in the visual word form area was also observed for fingerspelled words compared to one-handed signs. Body-anchored signs engaged bilateral superior parietal cortex to a greater extent than the descender baseline task and neutral space signs, reflecting the motor control and proprioceptive monitoring required to direct the hand toward a specific location on the body. Less activation in parts of the motor circuit was observed for two-handed signs compared to one-handed signs, possibly because, for half of the signs, handshape and movement goals were spread across the two limbs. Finally, the conjunction analysis comparing each sign type with the descender baseline task revealed common activation in the supramarginal gyrus bilaterally, which we interpret as reflecting phonological retrieval and encoding processes.



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Visual dorsal stream is associated with Chinese reading skills: A resting-state fMRI study

Publication date: September 2016
Source:Brain and Language, Volume 160
Author(s): Yi Qian, Yanchao Bi, Xiaosha Wang, Yi-Wei Zhang, Hong-Yan Bi
The present study explored the relationship between visual dorsal stream and Chinese reading by resting-state fMRI technique. We collected the resting-state brain activities and reading skills of Chinese-speaking adult readers. The results showed that the values of amplitude of low frequency fluctuation (ALFF) in right posterior parietal cortex (PPC) and left visual middle temporal area (MT) (two regions of dorsal stream) were significantly correlated with rapid naming (RAN) speed, and the ALFF values of right PPC were correlated with orthographic awareness (OA). Further resting-state functional connectivity (RSFC) analysis revealed that RAN speed was related to RSFCs between dorsal stream areas and reading areas (e.g., left fusiform gyrus, bilateral middle occipital gyrus). OA was correlated with RSFCs between right PPC and left middle occipital gyrus. It suggested that spontaneous activities of visual dorsal stream, as well as connection between it and reading-related areas, were highly associated with Chinese reading skills.



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Adjective conjunction as a window into the LATL’s contribution to conceptual combination

Publication date: September 2016
Source:Brain and Language, Volume 160
Author(s): Eva B. Poortman, Liina Pylkkänen
Though a large literature implicates the left anterior temporal lobe (LATL) for combinatory operations, recent MEG studies have suggested that it is specifically involved in the composition of complex concepts, rather than syntactic or semantic composition in a more general sense. To further specify the computational contribution of the LATL, we tested whether LATL effects as observed in MEG require a situation in which features combine to form a single coherent entity representation or whether the relevant computation simply requires the attribution of features to a set but not necessarily to the same members of the set. Under the former hypothesis, the LATL would be sensitive to the number of features added to the representation of a single entity whereas under the latter account, LATL activity would reflect the total number of features integrated across different members of a set. To test this, we employed conjunctions of two adjectives whose lexical semantics were varied such that they either allowed or disallowed the attribution of their denoted properties to the same members of a set, i.e., the properties were either compatible or incompatible. The compatible properties resulted in so-called intersective and the incompatible in so-called collective readings. Our results show that the LATL tracks the number of features attributed to an individual as opposed to the number of features attributed to a set. Interestingly, the reverse pattern was found in the right ATL, demonstrating that although this region often shows parallel effects to the LATL, its functional contribution is clearly distinct.



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Neural encoding of the speech envelope by children with developmental dyslexia

Publication date: September 2016
Source:Brain and Language, Volume 160
Author(s): Alan J. Power, Lincoln J. Colling, Natasha Mead, Lisa Barnes, Usha Goswami
Developmental dyslexia is consistently associated with difficulties in processing phonology (linguistic sound structure) across languages. One view is that dyslexia is characterised by a cognitive impairment in the "phonological representation" of word forms, which arises long before the child presents with a reading problem. Here we investigate a possible neural basis for developmental phonological impairments. We assess the neural quality of speech encoding in children with dyslexia by measuring the accuracy of low-frequency speech envelope encoding using EEG. We tested children with dyslexia and chronological age-matched (CA) and reading-level matched (RL) younger children. Participants listened to semantically-unpredictable sentences in a word report task. The sentences were noise-vocoded to increase reliance on envelope cues. Envelope reconstruction for envelopes between 0 and 10Hz showed that the children with dyslexia had significantly poorer speech encoding in the 0–2Hz band compared to both CA and RL controls. These data suggest that impaired neural encoding of low frequency speech envelopes, related to speech prosody, may underpin the phonological deficit that causes dyslexia across languages.



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Contextual modulation of hippocampal activity during picture naming

Publication date: August 2016
Source:Brain and Language, Volume 159
Author(s): A. Llorens, A.-S. Dubarry, A. Trébuchon, P. Chauvel, F.-X. Alario, C. Liégeois-Chauvel
Picture naming is a standard task used to probe language processes in healthy and impaired speakers. It recruits a broad neural network of language related areas, among which the hippocampus is rarely included. However, the hippocampus could play a role during picture naming, subtending, for example, implicit learning of the links between pictured objects and their names. To test this hypothesis, we recorded hippocampal activity during plain picture naming, without memorization requirement; we further assessed whether this activity was modulated by contextual factors such as repetition priming and semantic interference. Local field potentials recorded from intracerebral electrodes implanted in the healthy hippocampi of epileptic patients revealed a specific and reliable pattern of activity, markedly modulated by repetition priming and semantic context. These results indicate that the hippocampus is recruited during picture naming, presumably in relation to implicit learning, with contextual factors promoting differential hippocampal processes, possibly subtended by different sub-circuitries.



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Intrafamilial phenotypic variability of Specific Language Impairment

Publication date: August 2016
Source:Brain and Language, Volume 159
Author(s): Lisa Bartha-Doering, Sabrina Regele, Dirk Deuster, Rainer Seidl, Nadja Bogdanova, Albrecht Röpke, Peter Wieacker, Antoinette am Zehnhoff-Dinnesen
We investigated language functions in 32 members of a four generation family with several members affected by Specific Language Impairment with an extensive language test battery in order to determine the prevalence, overlap, and homogeneity of linguistic deficits within one pedigree. In sum, one fourth of all family members tested fulfilled the criteria of Specific Language Impairment. Despite of some similarities in language abilities, different combinations of language deficits were observed, and individual language profiles varied substantially. Thus, though there is a high prevalence of language deficits in this family which raises the likelihood of a genetic origin of these deficits, and though all affected study participants displayed selective linguistic deficits with normal non-verbal functioning, language testing showed considerable variance in overlap and homogeneity of linguistic deficits. Thus, even in one genetic population, an underlying linguistic disorder manifests itself in different language abilities to a variant degree.



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Examining the contribution of motor movement and language dominance to increased left lateralization during sign generation in native signers

Publication date: August 2016
Source:Brain and Language, Volume 159
Author(s): Eva Gutierrez-Sigut, Heather Payne, Mairéad MacSweeney
The neural systems supporting speech and sign processing are very similar, although not identical. In a previous fTCD study of hearing native signers (Gutierrez-Sigut, Daws, et al., 2015) we found stronger left lateralization for sign than speech. Given that this increased lateralization could not be explained by hand movement alone, the contribution of motor movement versus 'linguistic' processes to the strength of hemispheric lateralization during sign production remains unclear. Here we directly contrast lateralization strength of covert versus overt signing during phonological and semantic fluency tasks. To address the possibility that hearing native signers' elevated lateralization indices (LIs) were due to performing a task in their less dominant language, here we test deaf native signers, whose dominant language is British Sign Language (BSL). Signers were more strongly left lateralized for overt than covert sign generation. However, the strength of lateralization was not correlated with the amount of time producing movements of the right hand. Comparisons with previous data from hearing native English speakers suggest stronger laterality indices for sign than speech in both covert and overt tasks. This increased left lateralization may be driven by specific properties of sign production such as the increased use of self-monitoring mechanisms or the nature of phonological encoding of signs.



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Tracking the time course of lexical access in orthographic production: An event-related potential study of word frequency effects in written picture naming

Publication date: August 2016
Source:Brain and Language, Volume 159
Author(s): Qingqing Qu, Qingfang Zhang, Markus F. Damian
Previous studies of spoken picture naming using event-related potentials (ERPs) have shown that speakers initiate lexical access within 200ms after stimulus onset. In the present study, we investigated the time course of lexical access in written, rather than spoken, word production. Chinese participants wrote target object names which varied in word frequency, and written naming times and ERPs were measured. Writing latencies exhibited a classical frequency effect (faster responses for high- than for low-frequency names). More importantly, ERP results revealed that electrophysiological activity elicited by high- and low frequency target names started to diverge as early as 168ms post picture onset. We conclude that lexical access during written word production is initiated within 200ms after picture onset. This estimate is compatible with previous studies on spoken production which likewise showed a rapid onset of lexical access (i.e., within 200ms after stimuli onset). We suggest that written and spoken word production share the lexicalization stage.



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Neuroimaging genetic analyses of novel candidate genes associated with reading and language

Publication date: Available online 27 July 2016
Source:Brain and Language
Author(s): Alessandro Gialluisi, Tulio Guadalupe, Clyde Francks, Simon E. Fisher
Neuroimaging measures provide useful endophenotypes for tracing genetic effects on reading and language. A recent Genome-Wide Association Scan Meta-Analysis (GWASMA) of reading and language skills (N=1862) identified strongest associations with the genes CCDC136/FLNC and RBFOX2. Here, we follow up the top findings from this GWASMA, through neuroimaging genetics in an independent sample of 1275 healthy adults. To minimize multiple-testing, we used a multivariate approach, focusing on cortical regions consistently implicated in prior literature on developmental dyslexia and language impairment. Specifically, we investigated grey matter surface area and thickness of five regions selected a priori: middle temporal gyrus (MTG); pars opercularis and pars triangularis in the inferior frontal gyrus (IFG-PO and IFG-PT); postcentral parietal gyrus (PPG) and superior temporal gyrus (STG). First, we analysed the top associated polymorphisms from the reading/language GWASMA: rs59197085 (CCDC136/FLNC) and rs5995177 (RBFOX2). There was significant multivariate association of rs5995177 with cortical thickness, driven by effects on left PPG, right MTG, right IFG (both PO and PT), and STG bilaterally. The minor allele, previously associated with reduced reading-language performance, showed negative effects on grey matter thickness. Next, we performed exploratory gene-wide analysis of CCDC136/FLNC and RBFOX2; no other associations surpassed significance thresholds. RBFOX2 encodes an important neuronal regulator of alternative splicing. Thus, the prior reported association of rs5995177 with reading/language performance could potentially be mediated by reduced thickness in associated cortical regions. In future, this hypothesis could be tested using sufficiently large samples containing both neuroimaging data and quantitative reading/language scores from the same individuals.



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The peri-Sylvian cortical network underlying single word repetition revealed by electrocortical stimulation and direct neural recordings

Publication date: Available online 19 July 2016
Source:Brain and Language
Author(s): Matthew K. Leonard, Ruofan Cai, Miranda C. Babiak, Angela Ren, Edward F. Chang
Verbal repetition requires the coordination of auditory, memory, linguistic, and motor systems. To date, the basic dynamics of neural information processing in this deceptively simple behavior are largely unknown. Here, we examined the neural processes underlying verbal repetition using focal interruption (electrocortical stimulation) in 58 patients undergoing awake craniotomies, and neurophysiological recordings (electrocorticography) in 8 patients while they performed a single word repetition task. Electrocortical stimulation revealed that sub-components of the left peri-Sylvian network involved in single word repetition could be differentially interrupted, producing transient perceptual deficits, paraphasic errors, or speech arrest. Electrocorticography revealed the detailed spatio-temporal dynamics of cortical activation, involving a highly-ordered, but overlapping temporal progression of cortical high gamma (75–150Hz) activity throughout the peri-Sylvian cortex. We observed functionally distinct serial and parallel cortical processing corresponding to successive stages of general auditory processing (posterior superior temporal gyrus), speech-specific auditory processing (middle and posterior superior temporal gyrus), working memory (inferior frontal cortex), and motor articulation (sensorimotor cortex). Together, these methods reveal the dynamics of coordinated activity across peri-Sylvian cortex during verbal repetition.



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Neuroimaging correlates of language network impairment and reorganization in temporal lobe epilepsy

Publication date: Available online 5 July 2016
Source:Brain and Language
Author(s): S. Balter, G. Lin, K.M. Leyden, B.M. Paul, C.R. McDonald
Advanced, noninvasive imaging has revolutionized our understanding of language networks in the brain and is reshaping our approach to the presurgical evaluation of patients with epilepsy. Functional magnetic resonance imaging (fMRI) has had the greatest impact, unveiling the complexity of language organization and reorganization in patients with epilepsy both pre- and postoperatively, while volumetric MRI and diffusion tensor imaging have led to a greater appreciation of structural and microstructural correlates of language dysfunction in different epilepsy syndromes. In this article, we review recent literature describing how unimodal and multimodal imaging has advanced our knowledge of language networks and their plasticity in epilepsy, with a focus on the most frequently studied epilepsy syndrome in adults, temporal lobe epilepsy (TLE). We also describe how new analytic techniques (i.e., graph theory) are leading to a refined characterization of abnormal brain connectivity, and how subject-specific imaging profiles combined with clinical data may enhance the prediction of both seizure and language outcomes following surgical interventions.



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The Neural Mechanisms of Hallucinations: A Quantitative Meta-Analysis of Neuroimaging Studies.

The Neural Mechanisms of Hallucinations: A Quantitative Meta-Analysis of Neuroimaging Studies.

Neurosci Biobehav Rev. 2016 Jul 26;

Authors: Zmigrod L, Garrison JR, Carr J, Simons JS

Abstract
Activation likelihood estimation meta-analysis of functional neuroimaging data was used to investigate the neural mechanisms underlying auditory-verbal and visual hallucinations (AVHs and VHs). Consistent activation across studies during AVHs, but not VHs, in Wernicke's and Broca's areas is consistent with involvement of speech and language processes in the experience of hearing voices when none are present. Similarly, greater activity in auditory cortex during AVHs and in visual cortex during VHs supports models proposing over-stimulation of sensory cortices in the generation of these perceptual anomalies. Activation across studies in the medial temporal lobe highlights a role for memory intrusions in the provision of content for AVHs, whereas insula activation may relate to the involvement of awareness and self-representation. Finally, activation in the paracingulate region of medial prefrontal cortex during AVHs is consistent with models implicating reality monitoring impairment in the misattribution of self-generated information as externally perceived. In the light of the results, the need for unified theoretical frameworks that account for the full range of hallucinatory experiences is discussed.

PMID: 27473935 [PubMed - as supplied by publisher]

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Total Alloplastic Joint Reconstruction in a patient with temporomandibular joint (TMJ) ankylosis following condylar dislocation into the middle cranial fossa

Publication date: Available online 30 July 2016
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Ephraim R. Rikhotso, Muhammad A. Bobat




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Osteonecrosis of the Jaw in the Absence of Antiresorptive or Anti-angiogenic Exposure: A Series of Six Cases

Publication date: Available online 30 July 2016
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Tara L. Aghaloo, Sotirios Tetradis
Medication related osteonecrosis of the jaws (MRONJ) is a well-described complication of antiresorptive and antiangiogenic medications. Although osteonecrosis can be associated with other inciting events and medications such as trauma, infection, steroids, chemotherapy, and coagulation disorders, these are rarely reported in the literature. Since the continued development and clinical use of new biologic medications for diseases such as cancer and rheumatoid arthritis, it is important to continue to evaluate their effects on the oral cavity. Here, we report a six case series of MRONJ associated with medications other than antiresorptives or antiangiogenics. The degree of risk for osteonecrosis in patients taking these new classes of drugs is uncertain but warrants awareness and monitoring.



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Exophytic tumor growth after incomplete removal of polypoid malignant melanoma of the maxillary gingiva: A case report and review of the literature

Publication date: Available online 30 July 2016
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Tomoharu Taga, Taichiro Nonaka, Toshiaki Manabe, Kazuhisa Bessho
Polypoid malignant melanoma of the oral cavity is extremely rare. This report describes the case of three-time occurrence of polypoid malignant melanoma of the maxillary gingiva in an 84-year-old woman who had removed the primary tumor by herself. The second polypoid malignant melanoma was a black pedunculated mass measuring 7 cm in size and surrounded by pigmented mucosa. Histologically, the tumor exhibited an ulcerated surface lined by squamous cells and contained polygonal cells with brown–black pigments. The third polypoid malignant melanoma was observed at the same location 7 months after surgery; it was a black hemorrhagic mass approximately 1.5 cm in size. Histological analysis showed morphological findings that were similar to those observed in the second polypoid melanoma. The patient died of lung metastasis 28 months after the second surgery. This report also reviews the five previously reported cases of polypoid malignant melanoma of the oral cavity, all of which occurred in the upper jaw. In two cases, initial exophytic growth of the tumor before invasion of the submucosa and relatively early detection resulted in a good prognosis. However, in one case, amelanotic melanoma located in the periodontal tissues was clinically misdiagnosed as epulis. Therefore, immunostaining for S-100 and HMB-45 should be considered for non-pigmented, epulis-like lesions, and wide surgical resection of primary polypoid malignant melanomas at an early stage should result in a favorable prognosis.



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Loss of cargo binding in the human myosin VI deafness mutant (R1166X) increases actin filament binding.

Loss of cargo binding in the human myosin VI deafness mutant (R1166X) increases actin filament binding.

Biochem J. 2016 Jul 29;

Authors: Arden SD, Tumbarello DA, Butt T, Kendrick-Jones J, Buss F

Abstract
Mutations in myosin VI have been associated with autosomal recessive (DFNB37) and autosomal dominant (DFNA22) deafness in humans. Here, we characterise a myosin VI nonsense mutation (R1166X) that was identified in a family with hereditary hearing loss in Pakistan. This mutation leads to deletion of the C-terminal 120 amino acids of the myosin VI cargo-binding domain, which includes the WWY binding motif for the adaptor proteins LMTK2, Tom1 as well as Dab2. Interestingly, compromising myosin VI vesicle binding ability by expressing myosin VI with the R1166X mutation or with single point mutations in the adaptor binding sites leads to increased F-actin-binding of this myosin in vitro and in vivo As our results highlight the importance of cargo attachment for regulating actin binding to the motor domain, we perform a detailed characterisation of adaptor protein binding and identify single amino acids within myosin VI required for binding to cargo adaptors. We not only show that the adaptor proteins can directly interact with the cargo-binding tail of myosin VI, but our in vitro studies also suggest that multiple adaptor proteins can bind simultaneously to non-overlapping sites in the myosin VI tail. In conclusion, our characterisation of the human myosin VI deafness mutant (R1166X) suggests that defects in cargo binding may leave myosin VI in a primed/activated state with an increased actin-binding ability.

PMID: 27474411 [PubMed - as supplied by publisher]

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Predictors of Dysgeusia in Patients With Oropharyngeal Cancer Treated With Chemotherapy and Intensity Modulated Radiation Therapy.

Predictors of Dysgeusia in Patients With Oropharyngeal Cancer Treated With Chemotherapy and Intensity Modulated Radiation Therapy.

Int J Radiat Oncol Biol Phys. 2016 May 17;

Authors: Sapir E, Tao Y, Feng F, Samuels S, El Naqa I, Murdoch-Kinch CA, Feng M, Schipper M, Eisbruch A

Abstract
OBJECTIVE(S): Dysgeusia is a significant factor reducing quality of life and worsening dysphagia in patients receiving chemoradiation therapy for head and neck cancer. The factors affecting dysgeusia severity are uncertain. We investigated the effects on patient-reported dysgeusia of doses to the oral cavity, salivary output (required to dissolve food particles), and patient-reported xerostomia.
METHODS AND MATERIALS: Seventy-three patients with stage III to IV oropharyngeal cancer (OPC) (N=73) receiving definitive intensity modulated radiation therapy concurrently with chemotherapy participated in a prospective, longitudinal study of quality of life (QOL), including assessment of patient-reported gustatory function by taste-related questions from the Head and Neck QOL instrument (HNQOL) and the University of Washington Head and Neck-related QOL instrument (UWQOL), before therapy and periodically after treatment. At these intervals, patients also completed a validated xerostomia-specific questionnaire (XQ) and underwent unstimulated and stimulated major salivary gland flow rate measurements.
RESULTS: At 1, 3, 6, and 12 months after treatment, dysgeusia improved over time: severe dysgeusia was reported by 50%, 40%, 22%, and 23% of patients, respectively. Significant associations were found between patient-reported severe dysgeusia and radiation dose to the oral cavity (P=.005) and tongue (P=.019); normal tissue complication probability for severe dysgeusia at 3 months showed mean oral cavity D50 doses 53 Gy and 57 Gy in the HNQOL and WUQOL questionnaires, respectively, with curve slope (m) of 0.41. Measured salivary output was not statistically significantly correlated with severe taste dysfunction, whereas patient-reported XQ summary scores and xerostomia while eating scores were correlated with severe dysgeusia in the UWQOL tool (P=.04).
CONCLUSIONS: Taste impairment is significantly correlated with mean radiation dose to the oral cavity. Patient-reported xerostomia, but not salivary output, was correlated with severe dysgeusia in 1 of the 2 QOL questionnaires. Reduction in oral cavity doses is likely to improve dysgeusia.

PMID: 27473816 [PubMed - as supplied by publisher]

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Facial onset sensory and motor neuronopathy.

Facial onset sensory and motor neuronopathy.

Neurol Sci. 2016 Jul 29;

Authors: Zheng Q, Chu L, Tan L, Zhang H

Abstract
Facial onset sensory and motor neuronopathy (FOSMN) is a recently defined slowly progressive motor neuron disorder. It is characterized by facial onset sensory abnormalities which may spread to the scalp, neck, upper trunk and extremities, followed by lower motor neuron deficits. Bulbar symptoms, such as dysarthria and dysphagia, muscle weakness, cramps and fasciculations, can present later in the course of the disease. We search the PubMed database for articles published in English from 2006 to 2016 using the term of "Facial onset sensory and motor neuronopathy". Reference lists of the identified articles were selected and reviewed. Only 38 cases of FOSMN have been reported in the Pubmed database since it was first reported in 2006. Typically, FOSMN present with slowly evolving numbness of the face followed by neck and arm weakness. Reduced or absent of corneal reflexes and blink reflex is the main pathognomonic features of FOSMN. In this review, we summarize the epidemiology, clinical presentation, auxiliary examination, and treatment of all the reported cases of FOSMN. Moreover, we discuss the pathogenesis of this rare disorder. In addition, we propose diagnostic criteria for FOSMN.

PMID: 27473302 [PubMed - as supplied by publisher]

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