Dalmau Lab Questionnaire Version Date: 06/22/2015

Physician Information:
Referring Physician: Physician(s) Email:
Dr. Rubén Caparó rco@neuropediatrica.com

*Sample will not be processed without contact*

Patient Demographics:
Initials: C.R.A. Date of Birt Sex: Male Ethnicity: Native
(02/09/2010) x Female american

Sample Information:
☒CSF, Sample Date: 08/07/19 ☐Serum, Sample Date: ---

General Clinical Information:

Date of Disease Onset (30/06/2019):
Relevant Medical History (including background of autoimmunity):
- Three years diagnosis encephalitis autoimmune GLUR5.

Prodromal Symptoms (including headache, flu-like, etc.) or recent infections:

- It has featured troncal ataxia and focal seizure episode not associated with fictionation
duration less than 3 minutes of acute outburst.
- In addition to two day before the episode, convulsive or it a change in state, she looks weepy

Describe the clinical course (symptom presentation and progression):

Patient had 5 days of fever, before the onset of epileptic crisis. During 6th day of disease, he presents
with epileptic estatus, waking u
p after 8 hours. In 7th day of onset of disease, he presents with fever and refractory epileptic status, this
time he needs to be intubated and receive mechanical ventilation, he stays in the intensive care unit
for many days. He receives multiple antiepileptic drugs, endovenous human Immunoglobulin and
methylpdrenisolone, with slow favorable evolution. He currently has a diagnosis of refractory epilepsy
also. He has 3 studies of cerebrospinal fluid with normal cytochemical results, study for amoebas and
viruses with negative results.
Suspected Diagnosis:
Refractory Autoinmune encephalitis

SpecificClinicalInformation:
Pleaseelaborate on the clinical symptoms below (if present please explain)
Fever: Yes Movement disorders: Yes

*During neurological dysfunction, not explained by other causes *chorea, dyskinesia, abnormal posture, dystonia, orofacial
movements, myoclonus
Behavior/Psychiatric: Irritability Decreased Level of Consciousness: Yes

Cognition/Memory: Impaired Autonomic Instability: Yes

Speech Disorder: Yes Central Hypoventilation: No

Include a copy of this questionnaire with all shipments and send a copy via email to:
Lindsey.McCracken@uphs.upenn.edu&MARodes@clinic.ub.es
Dalmau Lab Questionnaire Version Date: 06/22/2015

Seizures/Status Epilepticus: Refractory status Sensory Deficits: No

epilepticus at beginning of disease. Now with
refractory epilepsy as well.

Sleep Disorder (insomnia/hypersomnia): Visual Impairment (unilateral/bilateral):

Insomnia No

Motor Impairment:Click here to enter text. Brainstem/Cerebellar: No

Reflexes: ☐ Increased
☐ Decreased
☒ Normal
Spinal Cord Symptoms: No

Tumor: No Other: Click here to enter text.

Test Results:
Test Date (mm/dd/yy) Result
CSF 02/05/19 Cells: 5
Glucose: 91 mg/dl
Protein: 23.6 mg/dl
Oligoclonal Bands: Not performed
Brain MRI 03/20/19 With contrast: In T2 and Flair, focal lesions y
*specify with/without contrast temporal lobes
Spinal Cord MRI 06/ /19 With contrast: Normal?
*specify with/without contrast
EEG 02/05/19 Pattern of disorganized basic rhythms, paroxysms
*specify slow, epileptic both of delta slow waves to frontal predominance

Treatment:
Summarize immunotherapy (including type, dates, and response):
IV steroids: Methylprednisolone 30 mg/Kg/day for five days and Human Immunoglobulin IV: 2 gr/kg
por five days, twice: 1° (Feb 2019) poor response, 2° (April 2019): Response is partially complete.
Continues with Prednisone.

Modified Rankin Scale (mRS):

Use the scale below to indicate the following
Date (mm/dd/yy) mRS Score
Worst Clinical State 02/06/19 5
Current Clinical State 06/24/19 2
List Remaining Deficits:Click here to enter text.

mRS 0 No symptoms at all

mRS 1 No significant disability despite some symptoms in clinical examination. Able to carry out all usual
activities (for children includes age appropriate behavior and further development)
mRS 2 Slight disability, unable to carry out all previous activities. Able to look after own affairs without
assistance (for children same independence as other age- and sex-matched children, without reduction
of levels on the gross motor function scale)

Include a copy of this questionnaire with all shipments and send a copy via email to:
Lindsey.McCracken@uphs.upenn.edu&MARodes@clinic.ub.es
Dalmau Lab Questionnaire Version Date: 06/22/2015
mRS 3 Moderate disability. Requires some help, but able to walk unassisted ; in younger patients adequate
motor development despite mild functional impairment (e.j. reduction of one level on the gross motor
function scale).
mRS 4 Moderately severe disability. Unable to attend to own bodily needs without assistance, and unable to
walk unassisted. (ej. in younger patients reduction of at least 2 levels on the gross motor function scale).
mRS 5 Severe disability. Requires constant nursing care and attention, bedridden
mRS 6 Dead

Include a copy of this questionnaire with all shipments and send a copy via email to:
Lindsey.McCracken@uphs.upenn.edu&MARodes@clinic.ub.es