Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder marked by chronic inflammation and progressive damage to the myelin sheath of peripheral nerves, causing muscle weakness, sensory loss, and diminished reflexes. It presents in various forms, including typical CIDP, distal acquired demyelinating symmetric neuropathy (DADS), and multifocal variants. Diagnosis involves clinical assessments, electrodiagnostic studies, and cerebrospinal fluid analysis, with imaging or nerve biopsy reserved for complex cases. Treatment focuses on immunomodulatory therapies like intravenous immunoglobulin (IVIg), corticosteroids, or plasmapheresis to manage symptoms and limit nerve damage.

CIDP affects 0.67 to 10.3 per 100,000 people, more often men, typically with onset between ages 40 and 60. While treatments are effective, challenges remain with adherence, relapses, and variable patient responses. Research is advancing with emerging biologics such as neonatal Fc receptor (FcRn) inhibitors and complement inhibitors. Prognosis varies, with some patients achieving remission, though ongoing management is crucial to prevent relapses and disability. Continued research aims to enhance diagnostics and develop therapies tailored to individual immune responses.

Reference: Gonzalez Caldito N, Habib AA. Diagnosis of chronic inflammatory demyelinating polyneuropathy. Practical Neurology (US). 2024;23(3):19-25.

Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder characterized by nerve cell loss and brain atrophy, often associated with abnormal accumulations of the tau protein. It typically begins around age 60 and progresses over six to eight years. Initial symptoms may affect one side of the body but eventually impact both sides, and include poor coordination, rigidity, memory loss, dementia, speech difficulties, and involuntary muscle jerks. As the disease progresses, individuals may develop difficulty swallowing, leading to complications such as pneumonia or sepsis, which are common causes of death. Due to symptom overlap with other diseases, such as Alzheimer's and Lewy body disease, CBD symptoms are now often referred to as "corticobasal syndrome."

There is no cure for CBD, and treatment primarily focuses on symptom management. Medications like clonazepam may help with muscle jerks, and therapies such as occupational, physical, and speech therapy can assist in managing daily challenges. Ongoing research continues to explore the complexities of CBD and its overlapping symptoms with other neurodegenerative diseases, aiming to improve diagnosis and treatment strategies.

Reference: Corticobasal Degeneration. Stanford Medicine. Accessed October 9, 2024. https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/corticobasal-degeneration.html

The Dementia with Lewy Bodies (DLB) Consortium has updated diagnostic criteria to improve detection and management, emphasizing clearer distinctions between DLB and Alzheimer’s disease (AD). While retaining the core structure of the 2005 criteria, the revisions better separate clinical features from biomarkers, now classified as indicative or supportive to enhance diagnostic precision. DLB’s key symptoms include cognitive fluctuations, visual hallucinations, parkinsonism, and REM sleep behavior disorder (RBD). Mixed pathologies, such as DLB with significant AD-like plaques, continue to complicate diagnosis.

Managing DLB requires a multifaceted approach due to its complex symptoms. Nonpharmacologic strategies, including exercise and cognitive training, help improve quality of life, while pharmacologic treatments like cholinesterase inhibitors address cognitive symptoms, and selective antipsychotics target psychiatric issues. Dopaminergic therapies, though less effective than in Parkinson’s disease, may worsen psychiatric symptoms. Future research will focus on refining diagnostics, exploring genetic and biomarker distinctions, and developing targeted treatments.

Reference: McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017 Jul 4;89(1):88-100. doi: 10.1212/WNL.0000000000004058. Epub 2017 Jun 7. PMID: 28592453; PMCID: PMC5496518.