portable-thermographic-screening-for-detection-acute-wallenberg-s

Portable Thermographic Screening for Detection of Acute Wallenberg's Syndrome

Wallenberg&#39;s syndrome (WS) is a type of brainstem infarction. WS patients often show Horner&#39;s syndrome, dissociated sensory disturbance, truncal ...

kanto Central Hospital

Reduced cardiac meta-iodobenzylguanidine (MIBG) uptake and loss of cardiac sympathetic axons, as its possible anatomical substrate, were both recognised in Lewy body disease (LBD), while their direct correlation has so far remained speculative. Increasing availability of autopsy-confirmed cases of LBD prompted us to quantify residual cardiac sympathetic axons to establish their relationship to cardiac MIBG uptake.

Quantitative correlation between cardiac MIBG uptake and remaining axons in the cardiac sympathetic nerve in Lewy body disease.

Autosomal-dominant striatal degeneration is a rare autosomal-dominant neurodegenerative movement disorder characterized by slowly progressive parkinsonism. Recently, a mutation of the cyclic nucleotide phosphodiesterase 8B gene was reported to be a causal gene mutation of this disease.

A novel mutation of PDE8B Gene in a Japanese family with autosomal-dominant striatal degeneration.

Teaching NeuroImages: The half-split man.

Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs. NIID has been considered to be a heterogeneous disease because of the highly variable clinical manifestations, and ante-mortem diagnosis has been difficult. However, since we reported the usefulness of skin biopsy for the diagnosis of NIID, the number of NIID diagnoses has increased, in particular adult-onset NIID. In this study, we studied 57 cases of adult-onset NIID and described their clinical and pathological features. We analysed both NIID cases diagnosed by post-mortem dissection and by ante-mortem skin biopsy based on the presence of characteristic eosinophilic, hyaline and ubiquitin-positive intanuclear inclusion: 38 sporadic cases and 19 familial cases, from six families. In the sporadic NIID cases with onset age from 51 to 76, dementia was the most prominent initial symptom (94.7%) as designated 'dementia dominant group', followed by miosis, ataxia and unconsciousness. Muscle weakness and sensory disturbance were also observed. It was observed that, in familial NIID cases with onset age less than 40 years, muscle weakness was seen most frequently (100%), as designated 'limb weakness group', followed by sensory disturbance, miosis, bladder dysfunction, and dementia. In familial cases with more than 40 years of onset age, dementia was most prominent (100%). Elevated cerebrospinal fluid protein and abnormal nerve conduction were frequently observed in both sporadic and familial NIID cases. Head magnetic resonance imaging showed high intensity signal in corticomedullary junction in diffusion-weighted image in both sporadic and familial NIID cases, a strong clue to the diagnosis. All of the dementia dominant cases presented with this type of leukoencephalopathy on head magnetic resonance imaging. Both sporadic and familial NIID cases presented with a decline in Mini-Mental State Examination and Frontal Assessment Battery scores. Based on these clinicopathological features, we proposed a diagnosis flow chart of adult-onset NIID. Our study suggested that the prevalence rate of adult-onset NIID may be higher than previously thought, and that NIID may be underdiagnosed. We should take NIID into account for differential diagnosis of leukoencephalopathy and neuropathy.

Clinicopathological features of adult-onset neuronal intranuclear inclusion disease.

Acute Wallenberg's syndrome (WS) is sometimes misdiagnosed as a nonstroke disease including auditory vertigo, and careful neurological examination is required for a precise diagnosis. Lateral difference of body surface temperature (BST) had been reported as a symptom of WS, although further details of this symptom are currently lacking. Our aim was to investigate the laterality of BST of patients with acute WS using thermography and the usefulness of thermography to detect acute WS.

Utility of thermographic measurements of laterality of body surface temperature to prevent misdiagnosis of acute Wallenberg's syndrome.

•Neutrophilic meningitis is rarely observed in Adult onset Still's disease (AOSD).•AOSD-related meningitis has been observed in the AOSD course of young adults.•An elderly man showed neutrophilic meningitis as a first symptom of AOSD.•First-line therapy with steroid for bacterial meningitis complicated the diagnosis.•Hyperferritinemia led to the correct diagnosis of AOSD and AOSD-related meningitis.

Article	Year
Quantitative correlation between cardiac MIBG uptake and remaining axons in the cardiac sympathetic nerve in Lewy body disease. Journal of neurology, neurosurgery, and psychiatry\| PubMed ID: 25935891	2015
A novel mutation of PDE8B Gene in a Japanese family with autosomal-dominant striatal degeneration. Movement disorders : official journal of the Movement Disorder Society\| PubMed ID: 26769607	2015
Teaching NeuroImages: The half-split man. Neurology\| PubMed ID: 27621385	2016
Clinicopathological features of adult-onset neuronal intranuclear inclusion disease. Brain : a journal of neurology\| PubMed ID: 27797808	2016
Utility of thermographic measurements of laterality of body surface temperature to prevent misdiagnosis of acute Wallenberg's syndrome. Brain and behavior\| PubMed ID: 29993195	2018
Adult-onset Still's disease presenting with aseptic meningitis as the first symptom in an elderly patient. eNeurologicalSci\| PubMed ID: 31485491	2019

Makoto Takahashi

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