Detection of underling proteinopathies is becoming increasingly important across neurodegenerative conditions due to upcoming disease intervention trials. In this review, we explored how temporal lobe changes in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) can potentially predict underlying TDP-43 pathology subtypes in FTD. To date, emphasis has been given to frontal lobe changes in the study of the cognitive and behavioural impairments in both syndromes but an increasing number of pathological, imaging and neuropsychological studies suggest how temporal lobe changes could critically affect the cognition and behaviour of these conditions. In this current article, we reviewed pathological, imaging as well as clinical/neuropsychological findings of temporal involvement in the ALS-FTD continuum, how they relate to temporal lobe changes and the underlying TDP-43 pathology in FTD. Findings across studies show that TDP-43 pathology occurs and coincides in many structures in ALS and FTD, but especially in the temporal lobes. In particular, anterior and medial temporal lobes atrophy is consistently found in ALS and FTD. In addition, memory and language impairment as well as emotional and Theory of Mind processing deficits that are characteristics of the two diseases are highly correlated to temporal lobe dysfunction. We conclude by showing that temporal lobe changes due to TDP-43 type B might be particular predictive of TDP-43 type B pathology in behavioural variant FTD, which clearly needs to be investigated further in the future.

Citations

• A comparative study of cognitive and behavioral profiles between sporadic and type 8 amyotrophic lateral sclerosis
  Cássia de Alcântara, Marcelo Maroco Cruzeiro, Marcondes Cavalcante França, Mariana Asmar Alencar, Antônio Jaeger, Caroline Martins de Araújo, Natália Araújo Sundfeld da Gama, Sarah Teixeira Camargos, Leonardo Cruz de Souza
  Muscle & Nerve.2023; 68(3): 316.     CrossRef
• In vivo exploration of synaptic projections in frontotemporal dementia
  Eric Salmon, Mohamed Ali Bahri, Alain Plenevaux, Guillaume Becker, Alain Seret, Emma Delhaye, Christian Degueldre, Evelyne Balteau, Christian Lemaire, André Luxen, Christine Bastin
  Scientific Reports.2021;[Epub]     CrossRef
• Structural and functional papez circuit integrity in amyotrophic lateral sclerosis
  Ana Paula Arantes Bueno, Walter Hugo L. Pinaya, Luciana M. Moura, Maxime Bertoux, Ratko Radakovic, Matthew C. Kiernan, Antonio Lucio Teixeira, Leonardo Cruz de Souza, Michael Hornberger, João Ricardo Sato
  Brain Imaging and Behavior.2018; 12(6): 1622.     CrossRef

Background: Meralgia paresthetica (MP) is a mononeuropathy affecting the lateral femoral cutaneous nerve. The disease is often diagnosed clinically, but electrophysiological tests play an important role. The aim of this study is to clarify clinical characteristics of MP as well as the role of sensory nerve conduction study (NCS) in the diagnosis of MP. Methods: Sixty-five consecutive patients with clinical diagnosis of MP between March 2001 and June 2012 were retrospectively reviewed at a single tertiary center. General demographics, clinical characteristics and sensory NCS findings were investigated. Measurements of sensory NCS included the baseline-to-peak amplitude, side-to-side amplitude ratio and the conduction velocity. To compare between the normal and abnormal NCS groups, independent t-tests and chisquare test were performed. Results: Sixty-five patients had male predominance (56.9%) with mean age of 48.4±13.4 years (range: 16-75). Seven patients (13.5%) had undergone operation or procedure before the symptom onset. The sensory nerve action potentials were obtainable in 52 (80%) of 65 clinically diagnosed MP patients. Sensory NCS revealed abnormalities in 38 patients (73.1%), and others (n=14, 26.9%) showed normal findings. Between the normal and abnormal NCS groups, there is no statistically significant difference on demographics or clinical features. Conclusions: We clarify the clinical features and sensory NCS findings of MP patients. Due to several limitations of sensory NCS, the diagnosis of MP could be accomplished both clinically and electrophysiologically.

Citations

• A cook with ‘burning in the thigh’ and a ‘hotspot’ in the groin!
  Wai Lun Moy
  Oxford Medical Case Reports.2021;[Epub]     CrossRef

Background
Meralgia paresthesia (MP) is characterized by sensory impairment in the anterolateral aspect of the thigh and usually caused by a lateral femoral cutaneous nerve (LFCN) lesion. It is well known that several physiologic factors including age, obesity, and sex can affect nerve conduction. This study aimed to determine whether body mass index (BMI) and age can influence on the conduction velocity and action potential amplitude of the LFCN.
Methods: Fifty six individuals without any previous neuromuscular disease participated in this study. LFCN was studied orthodromically,distally from the anterior superior iliac spine. The values, such as sensory nerve action potential (SNAP) amplitude and sensory nerve conduction velocity (NCV) were obtained. SNAP of the LFCN were formed on both sides in forty three individuals.
Results: No difference of demographic factors was observed between two groups divided according to the presence of SNAP formation. BMI had a significant relationship with SNAP amplitude and NCV of the LFCN. Moreover, Multiple regression analyses of nerve conduction values showed the significant correlation of body mass index and age with nerve conduction velocity.
Conclusions: We may suggest that nerve conduction of the LFCN can be affected by age and BMI. Further study to obtain normal nerve conduction data and compare these data with those of meralgia paresthetica patients should be continued.

Meralgia paresthetica (MP) is a benign entrapment neuropathy which is characterized by sensory impairment and paresthesia in the cutaneous distribution of the lateral femoral cutaneous nerve. A 79-year-old woman presented with intermittent right inguinal burning sensation. The sensory nerve conduction study (NCS) showed decreased right sidesensory nerve action potential (SNAP) on lateral femoral cutaneous nerve compared to the contralateral one. Abdomino-pelvic CT showed bilateral huge renal cysts (The size of largest one on right side: about 6.2

Memory in the brain is organized into multiple memory systems that perform different memory functions and have different neurologic substrates. The medial temporal lobe and midline diencephalic structures are essential in the establishment of new declarative memories and these memory traces are finally stored in domain-specific regions of the cerebral cortex. Nondeclarative forms of memory including skill learning, priming, and classic conditioning do not involve conscious recollection and rely upon the cerebral cortex, basal ganglia, and cerebellum.

Dysphagia consequent to neurologic diseases may be diverse depending on the nature and symptoms of the diseases. The initial step in the management of dysphagia would be systematic and through evaluation of swallowing function in both oral and pharyngo-laryngeal stages. Other important step might be to acquire knowledge on underlying neurologic disorder and its possible impact on swallowing. Specific indirect and direct management strategies that can be applied to specific impairments are presented.

We present a recordings of 37-year-old woman with simultaneous ictal scalp and subdural electrodes. The ictal rhythmon subdural electrocorticography (ECoG) started earlier (median 24.5 sec) and ended later (median 2.0 sec) compared toictal rhythm on scalp EEG. Eight ictal ECoG recordings were well localized to left temporal area, whereas ictal scalp EEGrecordings were not. Our case shows the obvious timing relations between two recordings, and different electrophysiologicinformation about localization of ictal onset zone.

Background
and Objectives: A nerve conduction study (NCS) has been known as a useful method to evaluate thetherapeutic effect of operation in carpal tunnel syndrome (CTS). To evaluate the temporal relationship between symptomaticand electrophysiological improvement, we compared the preoperative symptoms and electrophysiological resultswith postoperative those.Methods: We analyzed the NCS changes before and after minimal release of carpal tunnel in 26 patients (34 hands)with CTS. The time of postoperative symptomatic changes, postoperative electrophysiological changes and temporalrelationship between symptomatic and electrophysiological changes were evaluated.Results: The mean age was 49

We report a 29-year old female who developed bilateral femoral neuropathy combined with multiple abscesses in both thigh muscles. She was present with weakness in both lower extremities for 15 days and intermittent chilling sense for 3 months. Nerve conduction study showed complete absence of compound muscle action potentials in bilateral femoral nerves when stimulated at inguinal area. Electromyographic examination revealed no motor unit action potentials in both rectus femoris and vastus medialis muscles. CT revealed multifocal abscesses in bilateral thigh muscles. After antibiotic treatment, the patient's neurologic symptoms were improved.