Marchiafava-Bignami Disease
Description
● Definition / Overview:
• MBD is a rare neurological disorder associated with chronic alcoholism.
• Characterized by demyelination and necrosis of the corpus callosum.
• Pathognomonic imaging sign: “Sandwich sign” – central necrosis with peripheral gliosis.
● Case Details:
• Patient: 55-year-old male, chronic alcohol intake (~750 ml/day for 25 years).
• Presentation: Altered sensorium, loss of consciousness for 1 day.
• Clinical exam: Malnourished, E2V2M3 (Glasgow Coma Scale), no meningeal signs, normal pupils, no motor deficits.
• Laboratory findings: Normal hematology; liver function: ↑ gamma GT, bilirubin, ALP, ALT.
● Imaging Findings:
• MRI T1WI: Low signal in central genu, body, splenium of corpus callosum.
• MRI T2WI: High signal in central corpus callosum with sparing of dorsal/ventral layers → “Sandwich sign”.
• FLAIR: Hyperintense rim, hypointense core → central necrosis with gliosis.
• Other findings: Periventricular demyelination, demyelination in left frontal lobe.
• DWI / ADC: Early stage – hyperintense lesions with reduced ADC (cytotoxic edema); later stage – increased ADC (demyelination without axonal injury).
• MR Spectroscopy: ↑ choline, ↑ Cho/Cr ratio in acute phase; lactate peak in acute/subacute phase.
• SPECT: Bilateral reduction in cerebral blood flow.
● Clinical Classification:
➣Type A (Acute to Subacute):
•Impaired consciousness, pyramidal signs, limb hypertonia, seizures.
•Hyperintense swelling on T2WI. Poor prognosis.
➣Type B (Subacute/Chronic):
•Mildly impaired consciousness, dysarthria, gait disturbance, interhemispheric disconnection.
•Partial corpus callosum involvement on T2WI. Better prognosis; lesions may reverse.
● Differential Diagnosis:
•Multiple sclerosis (MS)
•Encephalitis
•Lymphoma
•Infarction (Recurrent artery of Heubner)
•Astrocytoma
•Progressive multifocal leukoencephalopathy
•Acute disseminated encephalomyelitis
● Clinical Forms:
➣Acute MBD: Seizures, impaired consciousness, rapid death.
➣Subacute MBD: Mental confusion, dysarthria, behavioral changes, memory deficits, gait impairment.
➣Chronic MBD: Mild progressive dementia over years.
● Treatment / Management:
➣Empirical therapy:
•Multivitamins (thiamine, B-complex)
•Corticosteroids
•Stabilization of plasma glucose
•Supportive care (hydration, nutrition, monitoring)
•Early diagnosis and prompt treatment are critical for potential reversal of early-stage lesions.
Protocol
● Medical Management (Difficulties and Solutions):
→ Arrhythmias
➣ Issue: Unusual electrical pathways causing irregular heartbeats.
➣ Treatment: Antiarrhythmic drugs (such as sotalol and amiodarone).
•Electrophysiology research to find aberrant pathways.
• Ablation of catheters to remove aberrant electrical circuits.
•In the event of severe arrhythmias, pacemaker insertion.
→ Heart Failure
➣ Issue: Dysfunction of the tricuspid valve causes right-sided heart failure.
➣ Treatment:
• Drugs: digoxin, ACE inhibitors, and diuretics.
• Tricuspid valve replacement or repair with surgery.
• Repair of related lesions of the atrial septum.
• Oxygen treatment in the event of hypoxia.
→ Infectious Endocarditis Risk
➣ Issue: Bacterial infection of the heart valves while undergoing surgery.
➣ Management:
• Prophylactic use of antibiotics before to surgery or dentistry.
• Pain, wound infection, and breathing problems following surgery are examples of postoperative complications.
➣Management: Consistent wound care and observation.
• Using analgesics to treat pain.
• Support for the respiratory system (breathing techniques, oxygen if required).
→ Electrophysiologic Issues
➣ Issue: Arrhythmias due to abnormal conduction.
➣ Management:
• Constant ECG observation.
• If necessary, surgical correction or catheter ablation.
● Nursing Interventions: Issues and Solutions
➣ Observation and Evaluation
• Keep an eye on your blood pressure, heart rate, and oxygen saturation levels.
• Monitor the ECG intermittently or continuously.
• Check for respiratory distress, cyanosis, and oedema, which are indicators of heart failure.
· Keep an eye on heart failure patients' fluid balance.
➣ Patient Education:
• Stress the value of taking medications as prescribed.
• Describe activity limitations in order to avoid overdoing it.
• Teach students to recognise warning symptoms, such as palpitations, exhaustion, and increasing shortness of breath.
➣ Postoperative Management
• Keep an eye out for infections at the surgery site.
• Administer and keep an eye on painkillers.
•Help with respiratory support and breathing exercises.
• Keep an eye out for issues like fluid excess or arrhythmias.
➣ Psychosocial Assistance
• Offer the patient consolation and emotional support.
• Provide family members with assistance and counselling.
• Offer tools for managing long-term disease.
Notes
For more information visit
10.7860/JCDR/2014/8377.4668
