Medical Criteria – N
NEUROFIBROMATOSIS (TYPE I; VON RECKLINGHAUSEN’S DISEASE)
- Diagnostic criteria include 2 or more of the following:
-
- More than 5 café-au-lait patches >1.5 cm in an adult or more than 5 café-au-lait macules >0.5 cm
- in a child <5 yr
- Axillary or inguinal freckling
- Iris hamartomas (Lisch nodules)
- Optic gliomas
- Neurofibromas
- distinctive bony lesion (sphenoid wing dysplasia or thinning of long bone cortex)
- First degree relative with neurofibromatosis Type 1
NEUROLOGICALLY DETERMINED DEATH:
- Criteria of Diagnosis
- Prerequisites: no CNS depressant drugs/neuromuscular blocking agents, no drug intoxication/ poisoning, temperature >32ºC, no electrolyte/acid-base/endocrine disturbance
- Absent brainstem reflexes: pupillary light reflex, corneal reflexes, oculocephalic response, caloric responses (e.g., no deviation of eyes to irrigation of each ear with 50 cc of ice water – allow 1 min after injection, 5 min between sides), pharyngeal and tracheal reflexes, cough with tracheal suctioning, absent respiratory drive at PaCO2 ≥60 mmHg, ≥20 mmHg rise above baseline, and pH ≤7.28 (apnea test)
-
- 2 evaluations separated by time, usually performed by two specialists (e.g., anaesthetist neurologist, neurosurgeon)
- confirmatory testing: Flat EEG, absent perfusion assessed with cerebral angiogram