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 Weakness and numbness in bilateral
lower extremities and unable to walk
 A 40 year old right handed legally blind female patient presented to the ER on
10/25/2014 with the chief complaint of Bilateral lower extremity weakness which
started a day before with flu like symptoms, back ache followed by tingling in the
right lower extremity and subsequently spread to the left lower extremity by next
morning with profound weakness and unable to use the lower extremities. Also
associated with bladder and bowel incontinence with bowel incontinence being new
to the patient. Uses crutches and power chair to ambulate at home but unable to
transfer from bed to chair since that day. Had a few falls in the process of
ambulation.
 History of similar episodes in the past since the age of 16years with relapses of
these symptoms at least 1-3 times an year. Her last episode was in May, 2014.
 At the age between 19-22 years old, she was paraplegic for several months and
became ambulatory after several months of physical therapy. Uneventful 2013 for
the patient with no flares and she delivered a healthy baby girl who is 8 months old
now.
 All her relapses in the past were dramatically improved with high dose IV steroids for
3-5 days with oral taper with some residual deficits after every episode.
 At baseline has urinary incontinence, stiffness in bilateral lower extremities with
spasms, patchy sensory loss in bilateral lower extremities.
 She became blind in her left eye at the age of 3yrs and only had central vision in her
right eye. She had symptoms of chronic urinary tract infections and was on
intermittant antibiotics because of the incontinence
 Recently had extraction of her wisdom tooth
 According to the patient this is the worst episode in many years which is different
from the rest of the flares by the rapidity of progression of symptoms, intensity of
symptoms, development of bowel incontinence and intensity of back pain.
Was a Neurology patient since the age of 19years when she was diagnosed and most of
her initial workup was done in Des Mois, Iowa city and used to follow Dr. Lardizabal, MD
in Northeast Regional Hospital since 2007 then here in the University Hospital till 2014
and now established care with Dr. Chuquilin, MD and may be a Friday conference case
in the past too.
 Ashthma
 GERD
 Neurogenic Bladder
 Recurrent UTIs

 Hypothyroidism
 B12 deficiency
 Polycystic Ovarian disease
Sister died at the age of 21 years, Blind since childhood, Unable to ambulate since the
age of 12-13 years and died of Pneumonia. Father died of Pancreatic cancer, Strong
family history of Lupus, Depression.
She lives with her daughter in Kirksville, MO. Doesn’t smoke, drink and denied any illicit
drug usage. Motivated to take care of her daughter, Didn’t work since childhood on
disability, Volunteered in the past in a Blind school in Iowa city. Had a Boyfriend in Iowa
city.
Allergic to Lovenox and Adhesive tape
 Advil 200 mg oral tablet, 2 Tablet(s) Oral q4h as needed, PRN: for fever
 Detrol LA 4 mg oral capsule, extended release
 Fish Oil: See Instructions, 1 tab bid
 Macrobid 100 mg oral capsule
 Prilosec: 20mg, Oral, Daily
 Singulair
 Vitamin B12 1000 mcg/mL injectable solution: 1 mg, 1 mL, IM, qMonth, 10 mL
 Vitamin B12 2500 mcg sublingual tablet: 2,500 mcg, 1 Tablet(s), Sublingual, Daily
 Vitamin D3 1000 intl units oral tablet
 Zantac 150
 albuterol: Inhalation
 baclofen: 20mg, Oral, bid
 brimonidine ophthalmic 0.2% solution
 cephalexin 500 mg oral tablet
 ciclopirox topical 8% kit:
 metformin: 500mg, Oral, bid
 Demyelinating disorders like Multiple Sclerosis/NMO/Transverse Myelitis
 Autoimmune polyneuropathies like AIDP, GBS(unlikely due to Bowel involvement),
Vasculitis.
 Infectious processes like Lyme’s disease, Tick paralysis, Sarcoidosis
 Thoracic myelopathy secondary to compression: Epidural abscess, Tumor, AVM
 Spinal cord infarction
 Vitals:
▪ Temperature (Celsius) 36.1 Deg C
 Heart Rate 84 bpm
 Respiratory Rate 20 breaths/min
 SBP NIBP 108 mmHg
 DBP NIBP 73 mmHg
 SpO2 100 %
 GENERAL EXAMINATION
 Patient in not apparent distress, cooperates with examination. Obese.
 HEAD: normocephalic, atraumatic, no lesions or exudates.
 EYES: normal.
 EARS, NOSE AND THROAT: normal, no lesions or exudates.
 NECK: supple, no lymphadenopathy or thyromegaly.
 EXTREMITIES: no clubbing, edema or cyanosis.
MENTAL STATUS:
 Patient was alert, awake and oriented x3, follows commands. Speech is fluent and comprehension is intact.
CRANIAL NERVES:
 II: Visual fields were full, Fundoscopic exam showed pale optic disc bilaterally. Pupils were reactive to light and
accommodation. APD bilaterally with no INO.
 III, IV, VI: Right eye exotropia. Nystagmus in all directions of gaze.
 V: face sensation was normal to light touch and pinprick.
 VII: face was symmetric. Eye closure and lip closure were normal.
 VIII: hearing is intact.
 IX-X: palate elevates at midline.
 XI: shoulder shrug is 5/5 bilaterally.
 XII: Tongue was midline and strong. No fasciculations.
MOTOR:
 Normal strength 5/5 on MRC scale in the upper extremities.
 Iliopsoas 0, hip abduction 0, hip adduction 1, quadriceps 2 on the right and 1 on the left, hamstring 2. Dorsiflexion
is 0 on the right side and 2 on the left side, plantar flexion is 0 on right side and 2 on the left side.
 Spasticity both lower extremities
 Fine finger movements were normal bilaterally. No pronator drift.
SENSATION:
 Decreased pinprick below rib cage level and both lower extremities.
 Vibration diminished in both the lower extremities up to knees. Strong on the right knee and absent on the left
knee
COORDINATION:
 Absent dysmetria on finger -nose -finger. Normal rapid alternating movements. No tremor.
GAIT:
 Wheelchair-bound
REFLEXES:
 3+ biceps, triceps, 4+ at the knees and ankles with sustained clonus. Babinski was positive bilaterally
 WBC – 9.0
 HGB – 14.1
 MCV – 87.8
 PLT – 284
 ESR – 23
 Electrolytes – Normal
 BUN – 11
 Creat – 0.72
 Vitamin B12 – 1124
 Folate - >40
 Vit – D – 26
 TSH – 0.425 with normal T4
 B2 – 23
 UA – negative
 Aquaporin-4 receptor antibody in serum – Later
Contrast
enhancement at
the level of T6
 >160 (Reference <4)
 Previous CSF studies done in 2007 in Northeast
Regional Hospital – Positive antibody, negative
Oligoclonal bands, IgG or pleocytosis. On Rebif since
2007, tried Copaxone and Avonex in the past.
Disease not well controlled. Patient is afraid of port
placement and refused Plasma exchange and IVIg
treatments in the past multiple times.
 Exam during the clinic visit is almost unchanged from
the hospital exam. Recommended Rituximab
infusions and Plasma exchange. She asked for 2
weeks of time hoping that steroids will work.
Devic's Disease
Devic's Disease
Devic's Disease
Devic's Disease
Devic's Disease
Devic's Disease
Devic's Disease

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Devic's Disease

  • 1.
  • 2.  Weakness and numbness in bilateral lower extremities and unable to walk
  • 3.  A 40 year old right handed legally blind female patient presented to the ER on 10/25/2014 with the chief complaint of Bilateral lower extremity weakness which started a day before with flu like symptoms, back ache followed by tingling in the right lower extremity and subsequently spread to the left lower extremity by next morning with profound weakness and unable to use the lower extremities. Also associated with bladder and bowel incontinence with bowel incontinence being new to the patient. Uses crutches and power chair to ambulate at home but unable to transfer from bed to chair since that day. Had a few falls in the process of ambulation.  History of similar episodes in the past since the age of 16years with relapses of these symptoms at least 1-3 times an year. Her last episode was in May, 2014.  At the age between 19-22 years old, she was paraplegic for several months and became ambulatory after several months of physical therapy. Uneventful 2013 for the patient with no flares and she delivered a healthy baby girl who is 8 months old now.  All her relapses in the past were dramatically improved with high dose IV steroids for 3-5 days with oral taper with some residual deficits after every episode.
  • 4.  At baseline has urinary incontinence, stiffness in bilateral lower extremities with spasms, patchy sensory loss in bilateral lower extremities.  She became blind in her left eye at the age of 3yrs and only had central vision in her right eye. She had symptoms of chronic urinary tract infections and was on intermittant antibiotics because of the incontinence  Recently had extraction of her wisdom tooth  According to the patient this is the worst episode in many years which is different from the rest of the flares by the rapidity of progression of symptoms, intensity of symptoms, development of bowel incontinence and intensity of back pain. Was a Neurology patient since the age of 19years when she was diagnosed and most of her initial workup was done in Des Mois, Iowa city and used to follow Dr. Lardizabal, MD in Northeast Regional Hospital since 2007 then here in the University Hospital till 2014 and now established care with Dr. Chuquilin, MD and may be a Friday conference case in the past too.
  • 5.  Ashthma  GERD  Neurogenic Bladder  Recurrent UTIs   Hypothyroidism  B12 deficiency  Polycystic Ovarian disease Sister died at the age of 21 years, Blind since childhood, Unable to ambulate since the age of 12-13 years and died of Pneumonia. Father died of Pancreatic cancer, Strong family history of Lupus, Depression. She lives with her daughter in Kirksville, MO. Doesn’t smoke, drink and denied any illicit drug usage. Motivated to take care of her daughter, Didn’t work since childhood on disability, Volunteered in the past in a Blind school in Iowa city. Had a Boyfriend in Iowa city. Allergic to Lovenox and Adhesive tape
  • 6.  Advil 200 mg oral tablet, 2 Tablet(s) Oral q4h as needed, PRN: for fever  Detrol LA 4 mg oral capsule, extended release  Fish Oil: See Instructions, 1 tab bid  Macrobid 100 mg oral capsule  Prilosec: 20mg, Oral, Daily  Singulair  Vitamin B12 1000 mcg/mL injectable solution: 1 mg, 1 mL, IM, qMonth, 10 mL  Vitamin B12 2500 mcg sublingual tablet: 2,500 mcg, 1 Tablet(s), Sublingual, Daily  Vitamin D3 1000 intl units oral tablet  Zantac 150  albuterol: Inhalation  baclofen: 20mg, Oral, bid  brimonidine ophthalmic 0.2% solution  cephalexin 500 mg oral tablet  ciclopirox topical 8% kit:  metformin: 500mg, Oral, bid
  • 7.  Demyelinating disorders like Multiple Sclerosis/NMO/Transverse Myelitis  Autoimmune polyneuropathies like AIDP, GBS(unlikely due to Bowel involvement), Vasculitis.  Infectious processes like Lyme’s disease, Tick paralysis, Sarcoidosis  Thoracic myelopathy secondary to compression: Epidural abscess, Tumor, AVM  Spinal cord infarction
  • 8.  Vitals: ▪ Temperature (Celsius) 36.1 Deg C  Heart Rate 84 bpm  Respiratory Rate 20 breaths/min  SBP NIBP 108 mmHg  DBP NIBP 73 mmHg  SpO2 100 %  GENERAL EXAMINATION  Patient in not apparent distress, cooperates with examination. Obese.  HEAD: normocephalic, atraumatic, no lesions or exudates.  EYES: normal.  EARS, NOSE AND THROAT: normal, no lesions or exudates.  NECK: supple, no lymphadenopathy or thyromegaly.  EXTREMITIES: no clubbing, edema or cyanosis.
  • 9. MENTAL STATUS:  Patient was alert, awake and oriented x3, follows commands. Speech is fluent and comprehension is intact. CRANIAL NERVES:  II: Visual fields were full, Fundoscopic exam showed pale optic disc bilaterally. Pupils were reactive to light and accommodation. APD bilaterally with no INO.  III, IV, VI: Right eye exotropia. Nystagmus in all directions of gaze.  V: face sensation was normal to light touch and pinprick.  VII: face was symmetric. Eye closure and lip closure were normal.  VIII: hearing is intact.  IX-X: palate elevates at midline.  XI: shoulder shrug is 5/5 bilaterally.  XII: Tongue was midline and strong. No fasciculations. MOTOR:  Normal strength 5/5 on MRC scale in the upper extremities.  Iliopsoas 0, hip abduction 0, hip adduction 1, quadriceps 2 on the right and 1 on the left, hamstring 2. Dorsiflexion is 0 on the right side and 2 on the left side, plantar flexion is 0 on right side and 2 on the left side.  Spasticity both lower extremities  Fine finger movements were normal bilaterally. No pronator drift. SENSATION:  Decreased pinprick below rib cage level and both lower extremities.  Vibration diminished in both the lower extremities up to knees. Strong on the right knee and absent on the left knee COORDINATION:  Absent dysmetria on finger -nose -finger. Normal rapid alternating movements. No tremor. GAIT:  Wheelchair-bound REFLEXES:  3+ biceps, triceps, 4+ at the knees and ankles with sustained clonus. Babinski was positive bilaterally
  • 10.  WBC – 9.0  HGB – 14.1  MCV – 87.8  PLT – 284  ESR – 23  Electrolytes – Normal  BUN – 11  Creat – 0.72  Vitamin B12 – 1124  Folate - >40  Vit – D – 26  TSH – 0.425 with normal T4  B2 – 23  UA – negative  Aquaporin-4 receptor antibody in serum – Later
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  • 17.  >160 (Reference <4)  Previous CSF studies done in 2007 in Northeast Regional Hospital – Positive antibody, negative Oligoclonal bands, IgG or pleocytosis. On Rebif since 2007, tried Copaxone and Avonex in the past. Disease not well controlled. Patient is afraid of port placement and refused Plasma exchange and IVIg treatments in the past multiple times.  Exam during the clinic visit is almost unchanged from the hospital exam. Recommended Rituximab infusions and Plasma exchange. She asked for 2 weeks of time hoping that steroids will work.