Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy in the same patient – a case report

Purpose: To investigate the clinical character, diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy accompanying myasthenia gravis so as to improve the understanding of such diseases.

Materials and methods: A case of chronic inflammatory demyelinating polyneuropathy combined with myasthenia gravis were analyzed retrospectively with review of the literature.

Results: This man was presented with chronic progressive sensory symptoms, flaccid tetraparesis, areflexia and protein-cell dissociation of cerebrospinal fluid. Nerve conduction study was indicative of demyelinating neuropathy. He was suspected as chronic inflammatory demyelinating polyneuropathy and treated with high-dose glucocorticoids. However, his condition worsened. Four months later, he was admitted and was diagnosed as combination of chronic inflammatory demyelinating polyneuropathy and myasthenia gravis. Good clinical results were observed after he was treated with pyridostigmine bromide, prednisone and mycophenolate mofetil.

Conclusions: This case warns clinicians to be aware of these two diseases presenting in the same patient, and the possible implications on treatment choices. A common immunological abnormality might exist in this rare association, but it still remains unknown.     

Behavioural and neural changes after a “choice” therapy for naming deficits in aphasia: preliminary findings

Background: Anomia, difficulty producing words, is a pervasive symptom of many individuals with aphasia. We have developed a treatment for naming deficits—the Phonological Components Analysis (PCA) protocol—that has proven efficacious in improving word-finding abilities for individuals with post-stroke aphasia.

Aims: The aim of this investigation is to present preliminary findings exploring the potential influence of choice—that is the active engagement of a participant in therapy—on our PCA treatment.

Methods & Procedures: Five individuals with aphasia were treated in one of two conditions—Choice or No Choice. Potential changes in neural activation as a function of the treatment were also investigated. Two individuals (one from each condition) underwent functional MRI (fMRI) pre- and post-therapy.

Outcomes & Results: All the individuals demonstrated a significant treatment effect immediately post-treatment and at a 4-week follow-up and four of the five participants at an 8-week follow-up. Three also demonstrated generalisation to untrained items. Unfortunately, no clear-cut patterns emerged to allow us to make claims about the influence of choice, per se, on the behavioural manifestations of improved naming. Interestingly, the participant from the Choice condition showed neural activation changes post-treatment in frontal and parietal regions that were not evident for the participant in the No Choice condition. Moreover, these changes were accompanied by a larger treatment effect for that individual and generalisation to a novel naming task.

Conclusion: The efficacy of PCA treatment for naming deficits is further supported. In addition, the neuroimaging data suggest the possibility that active engagement of an individual in his/her therapy (in this case choosing phonological attributes of a target word) may exercise executive functions important for success in treating anomia. Also, continued exploration of task factors that may promote even better treatment effects using this protocol is warranted, as is continued investigation of the neural underpinnings associated with treatment effects.     

Nummular headache in a patient with ipsilateral occipital neuralgia—A case report

Nummular headache (NH) is a rarely recognized primary headache, the diagnostic criteria of which are contained in the appendix to the 2nd edition of the International Classification of Headache Disorders (code A13.7.1).We present the case of a 61-year-old female who suffers, regardless of NH, from right-sided occipital neuralgia. The applied treatment – gabapentin and mianserin – had no effect. Injection of bupivacaine twice to the right occipital region resulted in neuralgia resolution up to three months, with no effect on NH.This confirms the independence of two abovementioned head pain conditions.     

Widespread microdysgenesis in therapy-resistant epilepsy – a case report on post-mortem findings

Microdysgenesis is a subtle malformation, which is often found in specimens from epilepsy surgery. It is, however, not clear whether the changes are focal or diffuse. A recent autopsy case offered an opportunity to investigate whether microdysgenesis found after temporal lobe surgery was focal or widespread in the brain. The entire brain of a 20-year-old patient who died suddenly and unexpectedly was examined histologically. Microdysgenesis had previously been diagnosed after a left temporal lobectomy performed because of therapy-resistant seizures. A light microscopic examination was performed on specimens stained with Luxol-fast blue-cresyl violet and polyclonal antibodies to glial fibrillary acidic protein. Widespread microdysgenesis with irregular nerve cell distribution in the cortex and an increased number of nerve cells in cortical layer I and in the white matter was found in the right temporal and parietal lobes and bilaterally in the frontal and occipital lobes. The post-mortem examination confirmed the previous diagnosis of microdysgenesis and showed that the changes were widespread in a patient who was operated on because of focal epilepsy.     

Compensating arithmetic ability with derived fact strategies in Broca’s aphasia: a case report

ABSTRACT

We investigated derived fact strategy use in RR, an aphasic patient with severely impaired working memory (no phonological loop), and 16 neurologically healthy matched controls. Participants were tested on derived fact strategy use in multi-digit addition, subtraction, multiplication, and division. RR’s accuracy only differed from controls in multiplication. He was as quick as controls in addition and subtraction when able to use the strategies, though significantly slower in addition, division, and multiplication without strategies. Our findings suggest the phonological loop is non-essential for multi-digit arithmetic, and derived fact strategies can help speed up arithmetic in individuals with impaired working memory.     

Could clomiphene kindle acute manic episode in a male patient? A case report

Psychiatric adverse effects associated with the use of clomiphene are relatively uncommon. Though case reports link mood swings to be associated with clomiphene, it is not known to be associated with a syndromal affective episode. Being a selective estrogen receptor modulator, clomiphene affects the hypothalamus–pituitary–gonadal axis and can have potential neuropsychiatric effects in vulnerable persons. Herein we report a case of clomiphene-induced manic episode in a known bipolar male patient.     

Visual disorders,the prosopometamorphopsia and prosopagnosia type in the early days after the onset of brain hemorrhagic stroke – a case report

Presented case report illustrates symptoms of prosopometamorphopsia (PM) and prosopagnosia, observed in the early days after the onset of a hemorrhagic stroke resulting from a complication of endovascular treatment of intracranial aneurysms and the use of anticoagulation therapy. PM is a visual disorder in which faces are perceived as distorted. The female patient described in the present study reported that faces she looked at seemed younger or older than in reality or as if they were dirty, swollen, or with a grimace. She also experienced symptoms of prosopagnosia, which is difficulty of recognizing familiar faces of people (e.g., of her husband and daughter). In the interview 6 months after the first examination, the patient reported spontaneous withdrawal of the visual disturbances.     

Frame aphasia due to Broca’s area impairment: a Persian case report

Background: Frame/content theory considers speech as a phenomenon evolved from mandibular cyclicities for the physiologic primitive functions of feeding.

Aims: This case report emphasises two aspects: first, the speech clinical characteristics of a left hemisphere damaged patient, which may be regarded as evidence of frame/content theory because there appears to be a regression to the most primitive stages of canonical babbling, and second, regarding its localised brain impairment limited to Broca’s area boundaries.

Methods & Procedures: We have provided a phonetically transcribed sample of speech and then analysed the articulated forms of syllables, mainly in words. A total of 403 syllables of the patient’s productions were analysed by articulatory phonetics method.

Outcomes & Results: Many patterns of articulation of this case, especially the labial–central and fronted dorsal–front pattern, may reveal a “frame without content” pattern that we believe to be a case of frame aphasia.

Conclusions: This patient may be considered as evidence of frame/content dissociation due to brain damage. Broca’s area’s role in subserving such a stage of content representation and loading in speech production is hypothesised.     

Do picture‐naming tests provide a valid assessment of lexical retrieval in conversation in aphasia?

Background: Word‐finding problems commonly occur in aphasia and can significantly affect communication. Assessment of this deficit typically involves naming pictures. However, this method has been criticised as lacking ecological validity. Alternative methods include the measurement of lexical retrieval in narration or conversation, although few published studies have quantified word finding in the latter.

Aims: We aimed to identify a reliable and valid assessment of lexical retrieval in conversation, and to elucidate the nature of the relationship between lexical retrieval in picture naming and in conversation.

Methods and Procedures: We developed a quantitative measure of word finding in conversation in aphasia and established the reliability and stability of the method. We compared the scores of a group of people with aphasia on this measure with their scores on a picture‐naming test.

Outcomes and Results: We found significant relationships between picture‐naming scores and a number of key variables analysed in the conversation measure. We propose that scores on picture naming relate to the ability to retrieve nouns in everyday conversation for the people with aphasia who took part in this study.

Conclusions: The use of picture‐naming tasks is justified, providing a valid and rich means of assessment of lexical retrieval. Further research is required to replicate these results with more people with aphasia. We offer the quantitative assessment of conversation developed here for use in research and clinical spheres.     

Recurrence of childhood Guillain-Barré syndrome after a long asymptomatic interval: a case report

We present a patient who rapidly developed flaccid paralysis in all 4 limbs on 2 separate occasions 19 years apart. Each episode was accompanied by a respiratory failure that required intubation. Both times, clinical response to immunotherapy was favorable with a near complete recovery. Clinical course, laboratory and electrodiagnostic findings, and favorable response to therapy suggest 2 separate attacks of Guillain-Barré syndrome. The reported case adds to a small series of cases of recurrent Guillain-Barré syndrome after a long asymptomatic period. In such cases, the distinction from chronic inflammatory demyelinating polyradiculoneuropathy should be made.     

Back to work with a chronic dysexecutive syndrome? (A case report)

Abstract

We present the case of G.L., a 33-year-old medical doctor, who, nine years after traumatic brain injury, was admitted to our department after he had drifted through several jobs. According to his superiors he spent too much time on routine activities and seemed unable to adapt himself to open problems and the requirements of novel or changing situations. From a psychometric perspective we found G.L. rather unremarkable, with largely good attentional, memory, and problem-solving performance. Behavioural analysis, however, revealed that G.L.'s discrete but definite frontal-lobe damage had produced defects in “executive functions”. After 12 months of intensive training G.L.'s mental structure had been modified to incorporate new source schemata, which enabled him to carry out demanding routine thought operations. In creating and practising these higher-level schemata his handicap could be diminished to the point that he now has got a supported employment. His basic cognitive incompetence, however, remained unchanged. This remarkable case fits in with Norman and Shallice's model of cognitive control of action and thought operations.