Significant inverse correlations were observed between BP values and AES-I-J scores in the left caudate nucleus and there was a marginally significant inverse correlation in the right caudate nucleus. The pathological basis of apathy might be the impairment of the dopaminergic nervous system. Further studies on more subjects with AD, healthy controls, and patients with PD and DLB are needed. The pathological basis of apathy might be the impairment of the dopaminergic nervous system. Further studies on more subjects with AD, healthy controls, and patients with PD and DLB are needed. Although various visual function deficits have been reported in patients with Alzheimer's disease (AD) and dementia with Lewy bodies (DLB), vegetable freshness perception has not been thoroughly examined. To investigate vegetable freshness perception in patients with AD and DLB and to clarify the relationship between vegetable freshness perception and various visuoperceptual functions. We enrolled 37 patients with probable DLB, 58 patients with probable AD, and 32 age-matched healthy controls. We assessed vegetable freshness perception and visuoperceptual functions, including vegetable brightness perception, contrast sensitivity, color perception, and stereopsis. Patients with DLB showed disproportionate deficits in vegetable freshness perception and vegetable luminance perception compared to patients with AD and controls. Analyses of the groups with higher and lower vegetable freshness perceptions revealed significant differences in contrast sensitivity and visual texture recognition. In the vegetable freshness test, we found significant differences among the 3 groups ( = 30.029, < 0.0001); the extent of impairment in patients with DLB was greater than that in patients with AD. In patients with DLB, the vegetable freshness judgments were significantly correlated with texture judgment scores and contrast sensitivity. Our findings revealed significantly impaired vegetable freshness perception in patients with DLB. Vegetable freshness perception may be related to visual texture recognition in patients with DLB. Our findings revealed significantly impaired vegetable freshness perception in patients with DLB. Vegetable freshness perception may be related to visual texture recognition in patients with DLB.Cervical spondylotic amyotrophy (CSA) is a rare clinical condition characterized by weakness and atrophy of the upper limb with minimal to no associated sensory deficits. The detection of the disease is based on clinical features at presentation, neurological examination, electrophysiological studies, and imaging. The proposed pathophysiological mechanisms include selective damage to the ventral root or anterior horn cells of the spinal cord. Depending on the muscle groups that are involved, CSA is broadly classified into a proximal type and a distal type. The clinical profiles of patients with CSA and ALS have a very close resemblance to each other, especially at the early stages of the disease. Cervical spine magnetic resonance imaging (MRI) may show T2 hyperintensity in both proximal and distal types. Electromyogram demonstrates denervation potentials and reduced motor unit potentials in the affected muscles. The conservative management is often the first-line modality, and those who fail to respond to conservative treatment have severe muscular atrophy and weakness, and distal-type CSA are considered potential candidates for surgery. We present the case of a 57-year-old female who presented with a 1-year history of left-hand weakness and wasting with no sensory deficits. https://www.selleckchem.com/products/orforglipron-ly3502970.html She denied any involvement of her other hand or bilateral lower limbs, and she was referred to our clinic with the potential diagnosis of amyotrophic lateral sclerosis (ALS). An elaborate history, physical examination, electrophysiological studies, and imaging assisted us in reaching the diagnosis of CSA, 1 year after the onset of symptoms.Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder featured by pontocerebellar dysfunctions and, in some cases, later cognitive disturbances. Here, we describe an atypical presentation of CLIPPERS, characterized by clinical onset with neuropsychiatric and cognitive symptoms. A 45-year-old man was referred to our Memory Clinic due to difficulties at work for over a month, caused by confusion and asthenia. Furthermore, insomnia and mood changes appeared. These disturbances were unresponsive to antipsychotic and antidepressant drugs. At admission, the patient presented also with severe cognitive impairment, urinary incontinence, ataxic gait, and limitation of lateral conjugate gaze. During the hospitalization, the patient underwent cerebrospinal fluid analysis, serum systemic autoimmune disorders laboratory research, neoplastic markers analysis, and brain MRI scan. The radiological and laboratory findings were compatible with the diagnosis of CLIPPERS. The sudden clinical and radiological improvement of the patient's conditions, after only a week of steroid therapy, further confirmed our clinical suspicion. The present case enhances the necessity to consider CLIPPERS in the differential diagnosis of pre-senile cognitive impairment, even in the absence of early pontocerebellar neurological signs. Before the spreading of the neuroinflammatory and degenerative processes, CLIPPERS represents one among the few possible reversible causes of cognitive decline.Alternating hemiplegia of childhood (AHC) (MIM 104290) is characterized by transient repeated attacks of paresis on either or both sides of the body, oculomotor and autonomic abnormalities, movement disorders, and cognitive impairment. Preventing paroxysmal attacks, such as paresis and spasm, in patients with AHC is often difficult. An 8-month-old girl presented to our institution with intractable epilepsy. She developed AHC, with left-right alternating or bilateral recurrent plegia upon waking, involuntary movements, eye movement abnormalities, and psychomotor retardation. She had a heterozygous de novo p.E815K mutation in the ATP1A3gene. Patients with this mutation develop severe hemiplegic spells and convulsions, have a poor neuromotor developmental outcome, and are particularly difficult to treat. Flunarizine treatment has limited therapeutic effect in such patients; however, it was definitely effective for bulbar palsy in the present case. The present case further highlights the need for the development of other new treatments, such as a ketogenic diet.