Unilateral left prosopometamorphopsia: A neuropsychological case study

We describe a patient who suddenly developed prosopometamorphopsia after a childbirth; she claimed that the left half of well-known and unfamiliar faces looked distorted. Brain MR was normal, whereas SPECT showed hypoperfusion of the left infero-lateral occipital cortex. No visual recognition defects for objects or faces were present. In three matching tasks with half-faces (Experiment 1), chimeric faces (Experiment 2), or chimeric objects (Experiment 3), the patient was impaired only when she matched pairs of chimeric faces differing in their left half; the same results were obtained after 1 year. This is the first behavioural demonstration of selective chronic metamorphopsia for the left side of faces, and provides new insights for models of face processing.     

Auditory perception in auditory neuropathy: clinical similarity with auditory verbal agnosia

The precise features of auditory perception in patients with auditory neuropathy have not been well described. In the present study, we examined auditory perception in a patient with auditory neuropathy. The patient was a right-handed 7-year-old boy. His chief complaint was delayed speech and suspected of verbal learning disability. He could talk, read and repeat rather fluently but could not understand fully what was asked. V-IQ, P-IQ and F-IQ of Wechsler Scale for Children III-R were 53, 118 and 81, respectively. Pure tone audiogram was completely normal. His speech discrimination ability was very poor. He could identify environmental sounds with visual matching. He could differentiate intensity difference but not time difference. This phenomenon was reported in patients with hemispheric symptoms. These clinical features are very similar to verbal auditory agnosia. ABR showed no response at 90dBnHL alternating clicks and tone bursts. Click evoked and distortion product otoacoustic emissions (OAE) were normal. Electrocochleogram was also normal. Motor and sensory nerve conduction velocity was completely normal. Pa of MLR and N1 of SVR were present. His diagnosis should be "pure type" of auditory neuropathy or auditory nerve disease. Importance of both ABR and OAE examination should be widely announced and auditory neuropathy must be campaigned stressed to be clinical entity among personnel who take care of children with speech delay.     

Central auditory processing disorders: review and case study

In this review paper, the prevalence and characteristics of central auditory processing disorder (CAPD) are reviewed. The causes, pathology and diagnosis of CAPD are also discussed. Recommendations are made for classroom interventions and a case study is presented. CAPDs are complex, heterogeneous disorders involving the process of auditory stimuli within the mechanisms of the central nervous system that can result in profound personal, social and academic consequences. Specificity in diagnosis, support by family members, neuroscience and allied health professionals and educators, and self-management and advocacy are required for children, adolescents and adults to have successful academic and work outcomes.     

Cerebellar pilocytic astrocytoma with auditory presentation: case study

An 8-year-old girl complained of decreased hearing and difficulty hearing from her right ear while on the telephone. Pure-tone and speech audiometry, immittance (tympanometry, acoustic reflex thresholds), auditory brainstem response, and transient click-evoked otoacoustic emissions were administered. The results were suggestive of a space-occupying lesion, and the patient was referred to a pediatric neurologist and neurosurgeon. A cerebellar pilocytic astrocytoma was found. The patient's audiologic profile is described, along with implications for pediatric neurologic evaluations.     

Pain in diabetic neuropathy case study: whole patient management

Painful diabetic peripheral neuropathy (DPN) is described as a superficial burning pain associated with other positive and/or negative sensory systems affecting the feet and lower extremities. It is one of the most commonly encountered neuropathic pain syndromes in clinical practice. Presentation may be complicated by multiple symptoms, including allodynia, hyperalgesia, other less well characterized dysesthesias, and serious disruption of social functioning and mood. Peripheral nerve function may deteriorate, which can account for patient reports of diminution of pain after several years of follow-up. Although current understanding holds that the pathogenesis of DPN is multifactorial in nature, long-term studies have shown that rigorous glycemic control is the most relevant factor in clinical intervention and can delay the onset and slow the progression of neuropathy. In addition to glycemic control, other treatment approaches must be examined in order to restore quality of life for patients experiencing painful DPN. Differential diagnosis is required to isolate DPN from other unexplained chronic pain. Neurologic testing in painful DPN is an area of active research and is used to assess the neurologic pathways giving rise to the pain, the degree of neural damage and the degree of subclinical damage. Current treatment options for DPN include mainly antidepressants and anticonvulsants, with other agents such as tramadol, dextromethorphan and memantine being employed or studied. This review article includes a case study of a patient with painful DPN to demonstrate the current management strategies for this neuropathic pain syndrome.     

Localization of callosal auditory pathways: a CT case study

The position of the auditory pathways in the human corpus callosum has not been defined by modern imaging techniques. We report a case with a discrete hemorrhagic lesion in the posterior body of the corpus callosum. The only signs of disturbed callosal function were limited to the auditory system--suppression of left ear stimuli with dichotic listening and neglect of left-sided auditory simultaneous stimuli.     

Progressive auditory neuropathy in patients with Leber's hereditary optic neuropathy

OBJECTIVE: To investigate auditory neural involvement in patients with Leber's hereditary optic neuropathy (LHON). METHODS: Auditory assessment was undertaken in two patients with LHON. One was a 45 year old woman with Harding disease (multiple-sclerosis-like illness and positive 11778mtDNA mutation) and mild auditory symptoms, whose auditory function was monitored over five years. The other was a 59 year old man with positive 11778mtDNA mutation, who presented with a long standing progressive bilateral hearing loss, moderate on one side and severe to profound on the other. Standard pure tone audiometry, tympanometry, stapedial reflex threshold measurements, stapedial reflex decay, otoacoustic emissions with olivo-cochlear suppression, auditory brain stem responses, and vestibular function tests were undertaken. RESULTS: Both patients had good cochlear function, as judged by otoacoustic emissions (intact outer hair cells) and normal stapedial reflexes (intact inner hair cells). A brain stem lesion was excluded by negative findings on imaging, recordable stapedial reflex thresholds, and, in one of the patients, olivocochlear suppression of otoacoustic emissions. The deterioration of auditory function implied a progressive course in both cases. Vestibular function was unaffected. CONCLUSIONS: The findings are consistent with auditory neuropathy-a lesion of the cochlear nerve presenting with abnormal auditory brain stem responses and with normal inner hair cells and the cochlear nucleus (lower brain stem). The association of auditory neuropathy, or any other auditory dysfunction, with LHON has not been recognised previously. Further studies are necessary to establish whether this is a consistent finding.     

Primary auditory neuropathy - an enigma

OBJECTIVES: To report audiological and neuroradiological findings in patients with primary auditory neuropathy (PAN) from a tertiary institution in south India. MATERIAL AND METHODS: The auditory profiles in 24 patients were analyzed along with neurological and radiological findings. RESULTS: Puretone audiometry showed varied hearing acuity and configuration. The syllable recognition score (n=15) was above 80% except in three. The dichotic digit test (n=11) showed bilateral suppression except in two who showed unilateral suppression. The auditory brainstem responses and the acoustic stapedius reflex were absent with distortion product otoacoustic emission (DPOAE) present in all. The neurological and radiological studies were normal. CONCLUSION: The audiological profile is consistent with the presence of DPOAE and absence of acoustic stapedius reflex and auditory brainstem responses. In patients with adequately preserved syllable recognition in quiet conditions, there was a significantly poor performance on dichotic digit test. As these patients had no neurological deficits and absence of specific etiological factors, we propose to use the term PAN for this entity.     

Cryoglobulinemic neuropathy: case report

We report the case of a 51-year-old man affected by mainly motor polyneuropathy with subacute onset and tendency to chronicization. Several possible etiological factors were present, but nerve biopsy, showing ischemic and inflammatory neuropathy, led us to consider cryoglobulinemia as the most important.

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Sommario Viene descritto il caso clinico di un uomo di 51 anni con polineuropatia principalmente motoria ad esordio subacuto e con tendenza alla cronicizzazione. La biopsia del nervo surale permette, evidenziando una neuropatia ischemica infiammatoria, di escludere il ruolo eziologico di altri concomitanti fattori di rischio, concludendo per una polineuropatia crioglobulinemica.

     

Unilateral isolated hypoglossal neuropathy associated to jugular paraganglioma

Neurological Sciences -     

Unilateral basal ganglia damage causes contralesional force control deficits: a case study

When grasping to lift an object, the grip force is usually scaled to the mass of the object. However, it has been shown that lifting objects of different sizes but equal masses results in the generation of higher forces for larger compared to smaller objects. The objective of this study was to investigate whether a similar effect is present in an individual (RI) with a unilateral lesion to the basal ganglia (BG). It was hypothesized that if the BG have an influence on the use of visual information in updating of the internal model used to anticipate the forces required for grasping, damage to these structures should result in the inability of RI's contralesional hand to anticipate object mass based on size cues. To test this hypothesis three objects of equal mass but different sizes were grasped and lifted by RI and six control individuals. The forces that were generated during these lifts were quantified. The controls showed the expected increases in peak grip force as object size increased. RI showed no effect of object size for his contralesional hand, but did show force scaling with his ipsilesional hand. In conclusion, RI's BG damage affected the on-line control of grip forces and the inability to integrate visual and tactile information in the programming of finger forces.     

Unilateral asterixis: case report and comments

Asterixis is usually bilateral. Occasionally, it may appear unilaterally. Hemiasterixis following infarction of the contralateral midbrain and thalamus is described. Mechanisms of asterixis and brain lesions associated with its unilateral appearance are reviewed. The mesodiencephalon is frequently implicated by the lesion either directly or indirectly, suggesting the importance of this structure in posture regulation.     

Auditory processing in individuals with auditory neuropathy

Background  

Auditory neuropathy is a disorder characterized by no or severely impaired auditory brainstem responses in presence of normal otoacoustic emissions and/or cochlear microphonics. Speech perception abilities in these individuals are disproportionate to their hearing sensitivity and reported to be dependent on cortical evoked potentials and temporal processing abilities. The disproportionate loss of auditory percept in presence of normal cochlear function is suggestive of impairment of auditory neural synchrony.     

Auditory processing in individuals with auditory neuropathy

Behavioral and Brain Functions (BBF) is an Open Access, peer-reviewed, online journal considering original research, review, and modeling articles in all aspects of neurobiology or behavior, favoring research that relates to both domains. Behavioral and Brain Functions is published by BioMed Central. The greatest challenge for empirical science is to understand human behavior; how human behavior arises from the myriad functions such as attention, language, memory and emotion; how these functions are reflected in brain structures and functions; and how the brain and behavior are altered in disease. Behavioral and Brain Functions covers the entire area of behavioral and cognitive neuroscience – an area where animal studies traditionally play a prominent role. Behavioral and Brain Functions is published online, allowing unlimited space for figures, extensive datasets to allow readers to study the data for themselves, and moving pictures, which are important qualities assisting communication in modern science.