Spontaneous spinal epidural hemorrhage following disseminated intravascular coagulation resulting in paraplegia: a case report.

CONTEXT: Spontaneous spinal epidural hemorrhage (SSEH) mostly presents as low back pain with or without a radiculopathy, and rarely with paraplegia or tetraplegia depending on the site and severity of spinal cord compression. We present here a case who had anemia and developed paraplegia following disseminated intravascular coagulation (DIC) due to a transfusion reaction. FINDINGS: A 65-year-old lady presented with sudden onset chest pain radiating to nape of the neck followed by loss of sensations and power in legs few hours after a blood transfusion. Her past history was negative for diabetes mellitus, hypertension, coronary artery disease, or a bleeding disorder. Her blood pressure was 90/57 mmHg and she had a normal pulse, respiratory rate, and temperature. On neurological examination, she had no motor power and unevokable muscle stretch reflexes in the lower limbs. The sensations were intact till T3 dermatome. The laboratory evaluation was suggestive of DIC. The magnetic resonance imaging showed a non-enhancing abnormal signal intensity area in the posterior epidural space, extending from CV4 to LV4 causing cervico-dorsal cord compression associated with cord edema. Following diagnosis, urgent decompressive surgery was carried out due to deteriorating neurological status. The patient was transfused with five bags of red cell concentrate, two bags of platelets, and four bags of fresh frozen plasma during the operation. The patient regained consciousness following operation, however, the neurological status did not improve. She, unfortunately, died on the third post-op day due to cardiac arrest. CONCLUSION: SSEH is a rare cause of paraplegia. Early radiological diagnosis is crucial for timely neurosurgical management and saving patient from permanent neurological deficit or a fatal outcome.

Familial Fahr's disease presenting with Parkinsonism in a young male.

Fahr's syndrome is a rare idiopathic neurodegenerative disorder which can present with wide spectrum of symptoms. It is characterized by bilateral symmetrical dystrophic intracranial calcification. It most commonly involves basal ganglia. A 30 years old male patient presented with progressive speech slurring and tremors of the right upper limb for last five years. His brain imaging studies revealed findings of extensive dystrophic calcification. He had an elder sister who had progressive limb weakness, tremors and speech slurring. Her brain imaging studies also showed same findings. On the basis of family history, neuroimaging findings and laboratory investigations, patient was diagnosed with the case of familial Fahr's disease. Fahr's disease should be considered as an important differential diagnosis in cases of Parkinsonism refractory to the treatment.