Q84 – 92: Central Nervous System

Questions 84 – 86: Was transcranial doppler velocity assessed at any time?

Transcranial doppler and transcranial color doppler are types of ultrasonography that measure the velocity of blood flow through the brain’s blood vessels by measuring the echoes of ultrasound waves moving trans-cranially.

Indicate if transcranial doppler velocity was assessed at any time in the current reporting period, independent of any CNS event that occurred. If Yes, report the date of the most recent assessment and the transcranial doppler velocity value in cm / sec. If transcranial doppler velocity was not assessed or if no information is available to determine if transcranial doppler velocity was assessed, report No or Unknown.

Question 87: Have any new central nervous system (CNS) complications occurred?

A CNS complication is the development of different neurologic signs and symptoms that occur in recipients with sickle cell disease. See Specify type of CNS complication for a list of CNS events.

Report Yes if the recipient experienced a new manifestation of a CNS complication(s) since the date of last report.

Report No in the following scenarios:

• A new CNS complication did not occur in the current reporting period.
• The recipient experienced a CNS complication in a prior reporting period and follow-up scans are performed in the current reporting period.

If no information is available to determine if a new CNS complication occurred since the date of last report, report Unknown.

**Multiple CNS Complications *
For each CNS complication the recipient experienced in the reporting period, complete questions Specify type of CNS complication, Year of onset, and Was an MRI / MRA of the brain performed for the diagnosis of this reported CNS complication? for each complication in the reporting period by adding an additional instance in the FormsNet3^SM application.

Question 88: Specify type of CNS complication

Indicate the type of CNS complication(s) that occurred. If multiple CNS complications occurred, report each CNS complication as a separate instance. If the same CNS complication occurred multiple times throughout the reporting period, only one instance needs to be reported.

• Cerebral venous thrombosis: A blood clot in the cerebral vein in the brain.
• Hemorrhagic stroke: Blood vessels break and bleeds in the brain.
• Ischemic stroke: The most common type of stroke. Occurs when a blood vessel in the brain is blocked or narrowed, causing reduced blood flow.
• Moyamoya: A rare condition where the carotid artery is blocked or narrowed which reduces the blood flow to the brain.
• Overt stroke: A focal neurologic deficit lasting more than 24 hours. If the type of CNS event is not documented and only noted as a “stroke,” select this option.
• Posterior reversible encephalopathy syndrome (PRES): An acute neurotoxic syndrome that is characterized by a spectrum neurological and radiological feature from various risk factors. Common neurological symptoms includes headache, impairment in level of consciousness, seizures, visual disturbances, and focal neurological deficits
• Seizure: Uncontrolled electrical activity in the brain, which may produce a physical convulsion, minors physical sings, thought disturbances or a combination of symptoms.
• Silent stroke: Asymptomatic stroke.
• Transient ischemic stroke: A temporary period of mild stroke symptoms that lasts only a few minutes and does not result in permanent damage. This is also known as transient ischemic attack or a ministroke.

Questions 80 – 90: Date of onset

Indicate if the date of onset of the new CNS event reported in Specify type of CNS complication is Known or Unknown. If Known, report the date of onset. If the date of onset is not known, select Unknown.

If the exact date is not known report an estimated date and check the Date estimated box. Refer to General Instructions, Guidelines for Completing Forms for information about reporting estimated dates.

Questions 91 – 92: Was an MRI / MRA of the brain performed for the diagnosis of this reported CNS event?

Magnetic resonance imaging (MRI) is an imaging technique used to form pictures of the anatomy and the physiological processes of the body. Magnetic resonance angiography (MRA) is similar to MRI but is used to specifically examine blood vessels.

If an MRI or MRA was performed to diagnose the reported CNS event, indicate Yes and attach a copy of the MRI / MRA report. Only attach the MRI / MRA report performed “at diagnosis” of the CNS event. The diagnostic MRI / MRA report may not be the most recent scan performed prior to the start of the preparative regimen. Do not attach MRI / MRA reports performed at any other time-point.

If an MRI / MRA was not performed to diagnose the reported CNS event or if no information is available to determine if an MRI / MRA was performed at diagnosis of the CNS event, report No or Unknown, respectively.

For instructions on how to attach documents in FormsNet3^SM, refer to the Training Guide.

Section Updates:

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