Median Nerve Stimulation in a Patient with Complex Regional Pain Syndrome Type II

A 54-year-old man experienced injury to the second finger of his left hand due to damage from a paintball gun shot 8 years prior, and the metacarpo-phalangeal joint was amputated. He gradually developed mechanical allodynia and burning pain, and there were trophic changes of the thenar muscle and he reported coldness on his left hand and forearm. A neuroma was found on the left second common digital nerve and was removed, but his symptoms continued despite various conservative treatments including a morphine infusion pump on his left arm. We therefore attempted median nerve stimulation to treat the chronic pain. The procedure was performed in two stages. The first procedure involved exposure of the median nerve on the mid-humerus level and placing of the electrode. The trial stimulation lasted for 7 days and the patient''s symptoms improved. The second procedure involved implantation of a pulse generator on the left subclavian area. The mechanical allodynia and pain relief score, based on the visual analogue scale, decreased from 9 before surgery to 4 after surgery. The patient''s activity improved markedly, but trophic changes and vasomotor symptom recovered only moderately. In conclusion, median nerve stimulation can improve chronic pain from complex regional pain syndrome type II.     

An autopsy case of frontotemporal lobar degeneration with the appearance of fused in sarcoma inclusions (basophilic inclusion body disease) clinically presenting corticobasal syndrome

We describe an autopsy case of basophilic inclusion body disease (BIBD), a subtype of frontotemporal lobar degeneration (FTLD) with the appearance of fused in sarcoma (FUS) inclusions (FTLD‐FUS), clinically presenting corticobasal syndrome (CBS). A 54‐year‐old man initially developed worsening of stuttering and right hand clumsiness. Neurological examinations revealed rigidity in the right upper and lower extremities, buccofacial apraxia, and right‐side dominant limb‐kinetic and ideomotor apraxia. Neuroimaging showed asymmetric left‐dominant brain atrophy and a cerebral blood flow reduction in the ipsilateral frontal region. At 56 years, his apraxia had advanced, and ideational apraxia was observed. Furthermore, the asymmetry in the limb‐kinetic and ideomotor apraxia had disappeared, and both conditions had become bilateral. He had a new onset of aphasia. His symptoms progressed and he died 9 years after the initial symptoms. The brain weighed 955 g. Diffuse brain atrophy was most obvious in the bilateral frontotemporal regions. The atrophy of the left superior frontal and precentral gyri and bilateral basal ganglia was remarkable. Histologically, there was a marked loss of neurons with gliosis in the affected areas, where basophilic neuronal cytoplasmic inclusions were observed. The inclusions were immunoreactive for FUS, p62, and TATA‐binding protein‐associated factor 15 (TAF15), but not for phosphorylated tau, transactive response DNA‐binding protein of 43 kDa (TDP‐43), neurofilament protein, or Ewing sarcoma (EWS). From these pathological findings, this case was diagnosed as having BIBD as an FTLD‐FUS variant. Spinal cord lower motor neurons were spared in number, similar to primary lateral sclerosis. Mutations in FUS were undetectable. Common background pathologies for CBS include corticobasal degeneration, Alzheimer's disease, PSP, FTLD with phosphorylated TDP‐43 inclusions (FTLD‐TDP), Pick's disease, Lewy body disease and CJD. However, FTLD‐FUS (BIBD) has been rarely reported. Our case suggested further pathological heterogeneity in CBS than had previously been reported. It is necessary to consider FTLD‐FUS (BIBD) as a background pathology for CBS in the future.     

Challenging behavior of patients with frontal dysfunction managed successfully with behavioral intervention

Herein, we report on two clinical cases in which behavioral disturbances occurred due to frontal dysfunction. Both patients were treated successfully with behavioral intervention. In the first case, the patient's challenging behavior of repeatedly entering his wife's workplace and interrupting her work was reduced after the patient's wife changed her response to the behavior and her schedule prior to work to ensure that she spent time with him in the morning. In the second case, the patient's challenging behavior of urinating in the street was reduced by changing his walking route. The successful outcome in both cases suggests that behavioral intervention based on Antecedents, Behavior, and Consequences (ABC) analysis is useful in the management of behavioral disturbance due to frontal dysfunction.     

Rapid Atypical Progression of Neuro-Beh?et's Disease Involving Whole Brainstem and Bilateral Thalami

We present a case of Neuro-Behçet''s disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient''s symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behçet''s disease with CNS involvement. The patient was treated with high-dose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behçet''s disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, low-grade glioma, multiple sclerosis, and occlusive venous disease.     

Giving up driving in Alzheimer's disease—an integrative therapeutic approach

For patients with Alzheimer's disease (AD), a recommendation to stop operating a motor vehicle can be a serious event complicated by a loss of self-esteem and personal dignity. Patients are often reluctant to give up an activity so essential, both practically and symbolically, to independent living. We describe here a patient with moderately progressed AD who lacked insight of his need to cease driving. Through an integrative treatment approach, combining behavioral and psychodynamic modalities, we helped him to formulate effective ways of coping with his loss of access to independent transportation. We favor a psychotherapeutic strategy that combines behavioral and managerial measures with dynamic patient interaction, thereby developing the patient's insight of the need to give up driving while fostering his sense of autonomy.     

Art and Parkinson’s disease: a dramatic change in an artist’s style as an initial symptom

In Parkinson's disease (PD), nonmotor symptoms manifest before motor symptoms. In this report, we present a remarkable case of a semiprofessional painter with PD whose painting style dramatically changed from abstract painting to realism before he developed motor, psychiatric, and autonomic nerve disorders. This case suggests that certain types of visual creativity may be impaired in the very early, presymptomatic stages of PD.     

Behavioral variant of frontotemporal dementia: Fundamental clinical issues associated with prediction of pathological bases

Behavioral variant of frontotemporal dementia (bvFTD) is a clinical syndrome characterized mainly by behavioral symptoms due to frontal dysfunction. Major neurodegenerative bases of bvFTD include Pick's disease, frontotemporal lobar degeneration with trans‐activation response DNA protein 43‐positive inclusions, corticobasal degeneration, and progressive supranuclear palsy. Early disinhibition characterized by socially inappropriate behaviors, loss of manners, and impulsive, rash and careless actions is the most important clinical feature of bvFTD. On the other hand, it was reported that clinical presentations of some Alzheimer's disease cases and patients with psychiatric disorders (e.g., addictive disorders, gambling disorder and kleptomania) often resemble that of bvFTD. Although clinical differentiation of ‘true’ bvFTD cases with frontotemporal lobar degeneration (FTLD) pathology from mimicking cases without it is not always easy, evaluation of the following features, which were noted in autopsy‐confirmed FTLD cases and/or clinical bvFTD cases with circumscribed lobar atrophy, may often provide clues for the diagnosis. (i) The initial symptoms frequently develop at 65 years or younger, and (ii) ‘socially inappropriate behaviors’ can be frequently interpreted as contextually inappropriate behaviors prompted by environmental visual and auditory stimuli. Taking a detailed history usually reveals various kinds of such behaviors in various situations in everyday life rather than the repetition of a single kind of behavior (e.g., repeated shoplifting). (iii) A correlation between the distribution of cerebral atrophy and neurological and behavioral symptoms is usually observed, and the proportion of FTLD cases with right side‐predominant cerebral atrophy may be higher in a psychiatric setting than a neurological setting. Finally, (iv) whether the previous course and the combination of symptoms observed at the first medical visit can be explained by major evolution patterns of clinical syndromes in pathologically confirmed FTLD cases should be considered. These views may provide clues to differentiate FTLD from Alzheimer's disease and to predict a subsequent clinical course and therapeutic interventions needed in the future.     

Mirtazapine improves visual hallucinations in Parkinson's disease: a case report

Psychotic symptoms often occur as a complication in Parkinson's disease patients, and a set of criteria for Parkinson's disease with psychosis (PDPsy) has been established. Among these criteria, hallucinations are one of the specific symptoms, with visual hallucinations being the most common. While atypical antipsychotic agents are often used for the treatment of PDPsy, adverse effects, including extrapyramidal symptoms, often hinder its continuation or tolerance. There have been some reports and reviews indicating that antidepressants may be effective for PDPsy and other forms of dementia with psychosis. In this report, we present a patient with PDPsy who was treated with one of the new‐generation antidepressants, mirtazapine. Mirtazapine improved the patient's refractory psychotic symptoms, especially her visual hallucinations, without worsening her motor symptoms.     

Intrinsic and extrinsic psychosis in Parkinson's disease

Direct and indirect signs and symptoms of Parkinson's disease are a major cause of disability in the elderly. Intrinsic symptoms comprise not only the well-known clinical hallmarks of this disease with motor behavioral abnormalities, such as bradykinesia, hypokinesia, rigidity and tremor, but also autonomic failure with orthostatic hypotension, urinal incontinence and impotence as well as non-motor behavioral abnormalities: mental dysfunction characterized by mood disorders, cognitive dysfunction and, sporadically, delusions and hallucinations. These symptoms are caused by a progressive abnormal degeneration of the dopamine (DA) producing cells in the substantia nigra (SN) and ventral tegmentum area (VTA) in combination with an interindividual fluctuating degree of decay in the noradrenergic (locus coeruleus), cholinergic forebrain (nucleus basalis of Meynert) and serotoninergic (dorsal raphe nuclei) systems. Extrinsic symptoms, induced by pharmacotherapy, mainly manifest with (un)predictable motor response fluctuations and dopaminomimetic psychosis. Psychological and psychiatric symptoms in Parkinson's disease (PD) are important predictors of the patient's quality of life. As these symptoms are potentially treatable, identification is of major clinical importance both for the patients and their caregivers and may enable to maintain Parkinson's disease patients at home for a longer period.

     

A case of Crow-Fukase syndrome with extramedullary plasmacytoma: marked clinical deterioration following a biopsy to plasmacytoma]

A 66-year-old man developed paresthesia of the distal parts of the bilateral lower limbs a week after his upper respiratory infection, followed by the weakness with the legs and paresthesia with the lip area, tongue and finger tips. Those symptoms gradually became worse to the point that he was unable to walk 10 days later. Although skin pigmentation, edema, and lymph node swelling were not found, we made a diagnosis of Crow-Fukase syndrome (CFS) because of clinical features of polyneuropathy, IgG-lambda type M proteinemia, endocrinological abnormality, elevated plasma level of vascular endothelial growth factor (VEGF) and extramedullary plasmacytoma in his abdomen. Following intravenous immunoglobulin therapy (IVIg), he showed marked improvement. However, his neurologic symptoms deteriorated acutely just after open biopsy together with the elevation of VEGF level, and a few days later he was in the state of flaccid quadriparesis. We tried IVIg therapy again and his neurologic symptoms were markedly improved. We speculated that an elevated VEGF, released from plasma cells induced by the bioprocedure, might have caused an increase in microvascular permeability and affected the blood-nerve-barrier, thereby his neurologic symptoms deteriorated. It is thought that this case may support the hypothesis that a significant role is played by VEGF in the pathomechanism of the development of CFS. Additionally we experienced that IVIg was very effective to the neurologic symptoms, and we think that IVIg will be able to be one of the future therapy of the CFS. To our knowledge, there has been no report of CFS which manifested acute deterioration of his neurologic symptoms just after open biopsy with acute onset with Guillain-Barré syndrome like symptoms.     

Charles Bonnet Syndrome Following Trans-Sphenoidal Adenomectomy without Optic Nerve Atrophy

Charles Bonnet syndrome (CBS) can develop after trans-sphenoidal adenomectomy (TSA); however, the neural mechanisms remain unknown. Sensory deprivation and releasing phenomenon are both hypothetical explanations for this condition; however, there is no definite evidence that strongly supports either supposition. We report the first case of CBS after TSA without optic nerve atrophy. Postoperatively, the patient''s vision seemed to be relatively well preserved, apart from the left-side hemianopsia in the right eye. Distinctive visual hallucinations only appeared when his eyes were closed, and these responded to quetiapine in a dose-dependent manner. Dose dependent change in colors and formation of hallucination was reported. Two weeks after quetiapine initiation, the patient''s CBS was completely resolved. This unique case suggests that blocking sensory input from the periphery is more critical than neural damage of the bottom-up connection to the visual association cortex. In addition, quetiapine should be considered as a specific treatment for CBS.     

Chondrosarcoma Apoplexy in Thoracic Spine

Chondrosarcoma is a very uncommon malignant primary bone tumor, especially, it occurs extremely rare in the spine. A 52-year-old man was admitted to the emergency room with sudden paraplegia. Twelve hours prior to a paraplegic event, he visited an outpatient clinic with discomfort and tenderness around the medial border of the right scapular, and his neurologic status was absolutely intact. Magnetic resonance imaging showed a lobulated soft tissue mass from T3 to T5, which extended to the epidural space. Computed tomography scans showed soft tissue mass on the spinal posterior arch and osteolytic change of the adjacent bony structures. Emergent surgery was performed and the lesion was removed. Dark reddish blood and gel-like material were encountered around the dura and posterior arch during the operation. Multiple pulmonary nodules were found on a chest CT scan and a biopsy of one of them had been proven to be a metastasis of chondrosarcoma. The histologic examination showed dedifferentiated chondrosarcoma. The patient''s neurologic deficit was improved slowly from ASIA A to ASIA D. Chondrosarcoma in the spine is extremely rare, even more with acute hemorrhage and sudden expansion into the epidural space. We named it chondrosarcoma apoplexy. We should consider the possibility of a hemorrhagic event when the patient''s neurologic deficit worsens suddenly with spinal bone tumor.     

Cognitive and behavioral profile in a case of right anterior temporal lobe neurodegeneration

Semantic dementia (SD) is a clinical variant of frontotemporal lobar degeneration (FTLD) characterized by progressive deterioration of semantic memory with relative sparing of other cognitive functions. It is associated with mainly left anterior temporal atrophy, and is also referred to as “left-temporal lobe variant” of FTLD. Recently, patients with mainly right-sided atrophy, or “right-temporal lobe variant”(RTLV), have been described. While some authors have reported that the initial and most significant deficit in these right-sided cases is a difficulty in recognizing famous people, others have observed that major behavioral abnormalities are the presenting symptoms. Here we report a detailed neuropsychological, language, behavioral and neuroimaging assessment of JT, a case of right temporal lobe variant of FTLD. JT showed early and prominent behavioral changes accompanied by a severe impairment in recognizing foods by their look, flavor or name. Later she also developed a difficulty in recognizing familiar people and objects. Standardized caregiver questionnaires of JT's pre- and post-morbid personality and interpersonal functioning showed that she went from being a flexible, dominant, extraverted, person to showing rigid, submissive and introverted behaviors. Her levels of neuroticism significantly increased, while her scores on agreeableness and cognitive and emotional empathy dropped. Voxel-based morphometry (VBM) showed most significant atrophy in the right amygdala/anterior hippocampal complex and collateral sulcus, extending to the right insula. We discuss the atypical cognitive and behavioral features of this case of RTLV of FTLD and stress the importance of behavioral changes and atypical semantic deficits for early diagnosis.     

Unusual clinical presentation and neuropathology in two subjects with fused‐in sarcoma (FUS) positive inclusions

We report two unusual autopsy cases with frontotemporal lobar degeneration (FTLD) that were hyperphosphorylated‐tau‐ and TAR DNA binding protein 43 (TDP‐43)‐ negative. The behavioral symptoms in both cases were compatible with frontotemporal dementia, but they exhibited more prominent speech and language related symptoms than previously reported. Moreover, they displayed a short duration of the disease; the male case had a disease onset age of 45 years, and duration of 5 years, and the female case suffered even shorter disease duration and a later onset of the symptoms, at the age of 67 years. Moreover, the motor functions had deteriorated in different ways in these cases. The male patient showed progressive motor symptoms, weakness of extremities and bulbar muscles suggesting motor neuron disease with a muscle biopsy supporting neurogenic deficits, whereas the female patient exhibited dyskinesias and tremor with progressive swallowing disorders. The father of the male case displayed dementia of similar type at the age of 68 years. In both cases, neuropathological examination showed fused‐in sarcoma (FUS)‐positive pathology. The male patient had intensely FUS‐positive cytoplasmic and intranuclear inclusions that resembled the characteristics previously reported in FTLD FUS, whereas the female patient did not exhibit any cytoplasmic inclusions but had roundish, dense FUS‐positive intranuclear inclusions. She also displayed a plethora of other pathologies including α‐synuclein, hyperphosphorylated‐tau, β‐amyloid aggregation and some neuronal polyglutamine aggregation (1C2) but no well‐demarcated inclusions were observed. We conclude that clinical phenotypes of FUS pathologies also include elderly patients and are more variable with motor and speech disorders than previously reported.     

Donepezil increases regional cerebral blood flow in a case of Korsakoff syndrome

Cholinesterase inhibitors improve cognitive functions and regional cerebral blood flow (rCBF) in mild to moderate Alzheimer's disease (AD). Recently, the possibility has been suggested that in patients with dementia other than AD cholinesterase inhibitors are useful for the treatment of their cognitive impairment and behavioral symptoms. In the present study we report the effectiveness of donepezil for a patient with Korsakoff syndrome. The patient was a 65‐year‐old man who gradually showed disturbances in memory, attention and concentration. The etiology was considered to be alcoholism. He would indulge in drinking and repeatedly asked the same questions over and over. He also developed emotional fluttering and would not settle down. On admission, he showed a severe memory deficit, disorientation and momentary confabulation. It was obvious that the patient was inactive and not spontaneous. He also manifested disinhibited behaviors. The patient's confabulation gradually diminished in hospital. He had no focal neurological deficit. Cranial magnetic resonance imaging (MRI) did not reveal any abnormality. Single photon emission computed tomography (SPECT) with 99 m‐TC‐ hexamethyl propylamenamine oxide (HMPAO) indicated hypoperfusion in the left thalamus, left basal ganglia and bilateral medial temporal lobes. In order to improve his cognitive function, the patient was treated donepezil. An evaluation of the effects of donepezil on rCBF was performed with the images showing a remarkable improvement in cerebral perfusion in the right medial temporal, the left thalamus and basal ganglia. However, the patient's memory impairment, disorientation, disinhibition, and stereotypical behavior and utterance were not improved. As an explanation, it was speculated that his neural network might be damaged so severely that there was no room for improvement by the acetylcholinesterase inhibitors.     

Clinical,Cognitive and Anatomical Evolution from Nonfluent Progressive Aphasia to Corticobasal Syndrome: A Case Report

Abstract

Recent clinical and pathological studies have suggested that frontotemporal lobar degeneration (FTLD) and corticobasal syndrome (CBS) show clinical and pathological overlap. We present four years of longitudinal clinical, cognitive and anatomical data in the case of a 56-year-old woman, AS, whose clinical picture evolved from FTLD to CBS. For the first three years, AS showed a progressive speech and language disorder compatible with a diagnosis of the nonfluent aphasia variant of FTLD. At year four, 10 years after her first symptom, AS developed the classical clinical signs of CBS, including alien limb phenomenon and dystonia. Voxel-based morphometry (VBM) applied to AS's four annual scans showed progression of atrophy from the inferior posterior frontal gyrus, to the left insula and finally to the medial frontal lobe. This case demonstrates the clinical overlap between FTLD and CBS and shows that the two can appear in the same patient at different stages of the disease in relation to the progression of anatomical damage.     

Does delay impair localisation in blindsight?

The unconscious sensorimotor skills which survive compromise of the geniculostriate visual pathway have been linked with activity of the dorsal stream of extrastriate occipitoparietal cortex. These sensorimotor circuits are thought to operate in real time. Therefore, an introduction of a delay between visual stimulus presentation and the patient's subsequent motor response should severely compromise sensorimotor tasks such as localisation (moving hand or eye to the location of a previously presented visual target). We tested this hypothesis in patient DB, a well-studied case of blindsight whose localisation abilities were first documented in the 1970s. Using eye tracking and hand movement recording technologies, as well as stimuli that control for light scatter, we verified the original observations of DB's manual and saccadic localisation. Remarkably, the introduction of a 4 s delay did not compromise his ability to localise with either eye or hand. A control experiment reveals that this skill does not depend on an opportunity to make a decision at the time of stimulus presentation, circumventing the delay using memory. These data suggest that DB's manual and saccadic localisation skills do not depend on the circuits of the dorsal stream, or that delay, contrary to theory, does not severely compromise dorsal sensorimotor skills.     

Acute Myelopathy Caused by a Cervical Synovial Cyst

Synovial cysts of the cervical spine, although they occur infrequently, may cause acute radiculopathy or myelopathy. Here, we report a case of a cervical synovial cyst presenting as acute myelopathy after manual stretching. A 68-year-old man presented with gait disturbance, decreased touch senses, and increased sensitivity to pain below T12 level. These symptoms developed after manual stretching 3 days prior. Computed tomography scanning and magnetic resonance imaging revealed a 1-cm, small multilocular cystic lesion in the spinal canal with cord compression at the C7-T1 level. We performed a left partial laminectomy of C7 and T1 using a posterior approach and completely removed the cystic mass. Histological examination of the resected mass revealed fibrous tissue fragments with amorphous materials and granulation tissue compatible with a synovial cyst. The patient''s symptoms resolved after surgery. We describe a case of acute myelopathy caused by a cervical synovial cyst that was treated by surgical excision. Although cervical synovial cysts are often associated with degenerative facet joints, clinicians should be aware of the possibility that these cysts can cause acute neurologic symptoms.     

Visual art in the neurologic career of Jean-Martin Charcot

Jean-Martin Charcot, the world's first chaired professor of neurology, incorporated visual art into his daily practice of neurology. Art served as scientific documentation and was a pivotal tool in the development and dissemination of Charcot's clinicoanatomic method. Although Charcot drew extensively in clinical and laboratory studies, very few of these visual documents have ever been published or are currently available for public study. Charcot was central to the incorporation of medical photographs into the study of neurologic disease and relied heavily on visual material in his capacity as an international teacher. Art also misguided Charcot's career when he relied heavily on artwork in his attempt to convince critics that disorders seen at the Salpêtrière Hospital, Paris, France, were independent of his suggestive influence.     

Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions

Although frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions (FTLD-TDP) and Pick’s disease are common pathological substrates in sporadic FTLD, clinical differentiation of these diseases is difficult. We performed a retrospective review of medical records and semiquantitative examination of neuronal loss of 20 sporadic FTLD-TDP and 19 Pick’s disease cases. Semantic dementia as the first syndrome developed only in FTLD-TDP patients. Impaired speech output in the early stage was five times more frequent in Pick’s disease than in FTLD-TDP. The total frequency of asymmetric motor disturbances (e.g., parkinsonism, pyramidal signs, and contracture) during the course was significantly more frequent in FTLD-TDP (78%) than in Pick’s disease cases (14%). Asymmetric pyramidal signs were found in 7 of 13 FTLD-TDP cases with corticospinal tract degeneration similar to primary lateral sclerosis. Frontotemporal dementia as the first syndrome was noted in both FTLD-TDP (28%) and Pick’s disease cases (64%); however, only FTLD-TDP cases subsequently developed asymmetric motor disturbances, and some of the cases further exhibited hemineglect. Concordant with these clinical findings, degeneration in the temporal cortex, caudate nucleus, putamen, globus pallidus, substantia nigra, and corticospinal tract was significantly more severe in FTLD-TDP, and degeneration in the frontal cortex tended to be more severe in Pick’s disease. Given these findings, the initial impairment of semantic memory or comprehension and subsequent asymmetric motor disturbances in sporadic FTLD patients predict sporadic FTLD-TDP rather than Pick’s disease, while initial behavioral symptoms or non-fluent aphasia without subsequent asymmetric motor disturbances predict Pick’s disease rather than sporadic FTLD-TDP. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.