Chronic Inflammatory Demyelinating Polyneuropathy with Reversible Dementia: A New Clinical Entity?
Notice bibliographique
Résumé
INTRODUCTION: Classic chronic inflammatory demyelinating polyneuropathy (CIDP), an acquired demyelination of peripheral nerves and nerve roots presents with symmetric motor and sensory involvement, weakness in proximal and distal muscles, globally diminished or absent reflexes, painful dysesthesias, and back pain with no brain involvement. In this case, a highly functional lawyer presents with reversible dementia and motor and sensory symptoms consistent with CIDP. This case may represent a new clinical entity of CIDP with reversible dementia. CASE REPORT: A 60-year-old man presented with progressive weakness, and cognitive dysfunction in the form of dementia over the last 8 weeks. Sensory and motor weakness continued to progress affecting upper and lower extremities with both proximal and distal muscle groups to the point where the patient was unable to move without assistance. The patient had word finding difficulty, short-term memory impairment, and was disoriented, despite his comprehension being intact. Initial Montreal Cognitive Assessment (MoCA) was 12/30. Initial neurologic exam was notable for muscle strength 3/5, globally depressed deep tendon reflexes. Lumbar puncture revealed elevated protein with no pleocytosis and no serum paraprotein. EMG/NCS demonstrated mixed sensorimotor axonal and demyelination peripheral polyneuropathy. CIDP was diagnosed based on clinical history according to Koski criteria. He was started on a 5-day treatment of IVIG, after which he had marked cognitive improvement after just one dose and improvement in weakness after the second dose of IVIG. Three weeks after IVIG treatment, the patient's cognitive function was back at baseline with MoCA score 29/30; no further word finding difficulty, and no short term memory impairment. At discharge, the patient's weakness had significantly improved to the point where he was able to walk with only the aid of a walker. His neurologic exam had improved as well as his muscle strength 4/5 and 2/4 deep tendon reflexes. Left sural nerve biopsy would eventually reveal moderate peripheral neuropathy with axonal degeneration, moderate loss of large and small myelinated nerve fibers, confirming diagnosis of CIDP. DISCUSSION: CIDP encompasses several different variants, including Lewis-Sumner syndrome, distal acquired demyelinating sensory neuropathy, sensory predominant CIDP among other variants. However, none of the variations of CIDP have a reversible cognitive impairment component. Patient met diagnosis of CIDP according to Koski criteria, as he had chronic polyneuropathy progressive for at least 8 weeks with no serum paraprotein, no genetic abnormality, and symmetric exam revealing weakness in all four limbs and proximal weakness in both lower extremities. Potentially, the patient could have had simultaneous diagnosis of dementia and CIDP, but the marked improvement in cognitive function after just one dose of IVIG makes that theory unlikely. PubMed search yielded no single case of CIDP with supratentorial manifestations in a patient with normal brain MRI. This case may represent a new clinical variant: CIDP with cognitive impairment.
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Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,001 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».