A case of multiple cranial nerve

lesions in a young lady

Sujaya Chattopadhyay
Arghyadeep Ganguly
Agrani Panda
 CONSENT

 Informed consent was obtained on the day of

admission after explaining the procedure to the
patient and her family in local language.
 PATIENT PARTICULARS
 Name: Mallika Mondal
 Age: 20years
 Sex: Female
 Address: Murshidabad
 Religion: Hindu
 Occupation: Housewife
 Date of Admission: 25th July, 2019
 Dates of Examination: 25th July, 2019; 9th August, 2019
 CHIEF COMPLAINTS

 Headache for 1 month

 Double vision for 15 days

20 year old woman was admitted to our college on the 28th of

July with the above chief complaints. She was apparently well
till her last delivery 1 month ago that resulted in spontaneous
abortion.
 HISTORY OF PRESENT ILLNESS
The headache had the following features:
 Insidious in onset
 Diffuse
 Deep seated
 Throbbing in nature
 Persistent throughout the day and night
 Associated with occasional vomiting and disturbed sleep.
 Relieved partially by taking over-the-counter medications

Not associated with redness of eyes, lacrimation, running nose, aural fullness
 HISTORY OF PRESENT ILLNESS (Contd.)
Patient also complained of double vision 15 days before
admission to hospital which was perceived on looking to both sides.

After admission, within 4 days, patient developed complete

drooping of the eyelid on the right side(with disappearance of diplopia).

After 9 days of admission, patient developed rapid onset of painless dimness of

vision, which started in right eye followed by involvement of left eye within 3
days resulting in complete blindness.

In the hospital, the eye movements as well as the drooping of the eyelid had
gradually improved, however the vision had not improved in either eye.
HISTORY OF PRESENT ILLNESS (Contd.)
She also complained of weakness of right lower limb 14
days after admission. This was
 Acute in onset
 Non progressive
 Without associated abnormal movements , stiffness or
twitching of muscle
 No sensory involvement (pain, touch, temperature), no
bowel and bladder dysfunction.
HISTORY OF PRESENT ILLNESS (Contd.)
There was no history of

 Other Cranial nerve involvement

 Joint pain
 Prolonged fever
 Oral ulcers
 Skin rash
 Convulsions
 Loss of consciousness
 HISTORY OF PAST ILLNESS

 Around 1 year ago ,she had suffered from an episode of

unilateral painful calf swelling which subsided with
compression stockings.
 She had no history of similar neurological illness in the
past
 She had no contact history of tuberculosis.
TREATMENT HISTORY
 She was not on any long term medication.
 She received inj. methylprednisolone & inj. Heparin
during the hospital stay.
 MENSTRUAL AND OBSTETRIC HISTORY
 Menstrual History- Normal
 Obstetric History: She had a history of 3 consecutive,
spontaneous, fetal losses:
 1st in 2017 at 8 weeks,
 2nd in 2018 at 12 weeks and
 3rd in 2019 at 20 weeks of gestation
Married for 4 years with no living issue
OTHER HISTORY
 Personal history- No history of any allergies, addictions;
Normal bowel, bladder, appetite. No significant
weight loss.

 Family History- No family History of Diabetes, Hypertension, Collagen

Vascular disease, Cardiovascular Disease ,any blood disorder were
noted. There was no case of TB or meningitis in close contacts.

 Socio-economic History- Patient lived in kuchha house and belonged

to Class IV according to the modified BG Prasad scale.

 Psychiatric history- No abnormalities detected

GENERAL SURVEY (on day 16)
 Pulse- 78/min, regular rhythm, low volume, no delay, no
special character, arterial wall not palpable
 Respiration: 28/min, regular, thoraco-abdominal
 Blood Pressure- 110/70 (rt arm supine)
 Temperature- normal
 Neck lymph nodes- Not palpable
CENTRAL NERVOUS SYSTEM EXAMINATION
CRITERIA ON ADMISSION ON DAY 16

Higher functions Confused GCS- 15/15

GCS-11/15
Spine and cranium Normal Normal

Meningeal signs Normal Normal

Sensory system Normal Normal

Autonomic Normal Normal

Cerebellar Normal Normal
Gait Normal Unable to stand without support
CENTRAL NERVOUS SYSTEM EXAMINATION (Contd.)
CRANIAL ON ADMISSION ON DAY 16
NERVE Right Left Right Left
I Normal Normal Normal Normal
II Papilledema- present LP- absent,
Papilledema- present
III, IV,VI LR palsy + LR palsy + Complete LR palsy +
Rest-N Rest-N ophthalmoplegia

Pupils normal Pupils bilaterally dilated, NR to light

V Normal Normal Normal Normal
VII Normal Normal Normal Normal
VIII Rinne-N Rinne-N Rinne-N Rinne-N
Weber-N Weber-N Weber-N Weber-N
IX, X Normal Normal Normal Normal

XI, XII Normal Normal Normal Normal

CENTRAL NERVOUS SYSTEM EXAMINATION (Contd.)

 The abnormal findings were confined to the right lower

limb.
Motor System On admission On day 16
examination
Atrophy Absent Absent
Abnormal movements Absent Absent
Tone Normal Hypotonia in right lower
limb
CENTRAL NERVOUS SYSTEM EXAMINATION (Contd.)
 The abnormal findings were confined to the right lower
limb.
FEATURE On admission At present

Power Normal Grade 3

Co-ordination Present Present

Jerks
• Knee Normal Diminished in right lower
limb
• Ankle Normal Diminished in right lower
limb
• Plantar reflex Flexor Equivocal
OTHER SYSTEMS

 Cardiovascular system: No significant findings

 Respiratory system: No significant findings
 Gastro-intestinal system: No significant findings
 POSSIBILITIES
 APLA syndrome
 Vasculitis
 NMO
 Multiple Sclerosis
 Paraneoplastic syndrome
INVESTIGATIONS
ROUTINE INVESTIGATIONS
Hb- 10.9 g/dl
TLC- 17,800/ cu.mm ( N70 L24 M3 E3 )
PLATELET- 40,000/ cu.mm
ESR- 60 mm/hr
ROUTINE INVESTIGATIONS (Contd.)
 Blood sugar (fasting): 91 mg/dl
 Serum electrolytes- Normal
 Serum urea- 37mg / dl
 Serum creatinine- 1.0mg/dl
 Chest X-Ray- Normal
 ECG- normal
 LFT- normal
 Urine examination- normal
 CSF Examination- Normal
SPECIAL INVESTIGATIONS
OPHTHALMOSCOPY AND VEP:

Optic disc- margin blurred

Retina- normal
Retro- orbital pathology
excluded.
No scotoma , no hemianopia
SPECIAL INVESTIGATIONS (Contd.)
CT Brain (25th July, 2019)
SPECIAL INVESTIGATIONS (Contd.)
MRI Brain (25th July, 2019)
SPECIAL INVESTIGATIONS (Contd.)
MR Venogram (2nd August, 2019)
SPECIAL INVESTIGATIONS (Contd.)
MR Angiogram (2nd August, 2019)
SPECIAL INVESTIGATIONS (Contd.)
MRI Orbits
(8th August,2019)
SPECIAL INVESTIGATIONS (Contd.)
Spinal Screening MRI (8th August, 2019)
 SPECIAL INVESTIGATIONS (Contd.)
COAGULATION PROFILE
 APTT- 43 seconds
 PT/INR: 13.2 seconds; INR- 1.2
 Protein C- 129.76
 Anti-thrombin III- 78%
 Factor V Leiden mutation detection- Negative
SPECIAL INVESTIGATIONS (Contd.)
APLA PROFILE
 Anti-cardiolipin antibody: IgG-19 PL-U/ml
IgM-9.01 PL-U/ml
 Anti B2 -glycoprotein antibody-
IgG- 56.61 RU/ml
IgM- 120.37 RU/ml
 Lupus anticoagulant: 31 s
SPECIAL INVESTIGATIONS (Contd.)
AUTOIMMUNE MARKERS
 ANA: 42.8 IU/ml
 p-ANCA = 1.571 AU/ml
 c-ANCA= 1.377 AU/ml

 ENA profile: Normal

 Complement C3: 109 mg/dl
 Complement C4: 17.2 mg//dl
 Direct Coombs’ test: Negative
 SPECIAL INVESTIGATIONS (Contd.)
OTHER RELEVANT SEROLOGIES
 Anti-aquaporin 4 antibody -
Negative
 CSF oligoclonal band- Absent
 Anti-Ri- Negative
 Anti-amphiphysin- Negative
 CONCLUSION
 A 20 year old lady, non diabetic, complained of headache since 1 month and double
vision since 15 days along with onset of monoparesis after admission. It was
accompanied by ptosis of the right eyelid followed by complete blindness in both
eyes and a past history of 3 consecutive ,spontaneous fetal losses .
 Examination revealed II, III, IV, VI nerve palsy in right eye and II,VI nerve palsy in left
eye.There was hypotonia of the right lower limb along with diminished jerks and
an equivocal plantar response.
 Investigations revealed thrombocytopenia, blurred optic disc margins with no
retro-orbital pathology. A thrombosis was detected at the posterior aspect of right
superior sagittal sinus along with hemorrhagic transformation at the right
temporo-parietal region. The APTT was in the high normal range. In the serology
profile, a high titer of IgM B2- glycoprotein was observed. The rest of the
radiological investigations, coagulation studies and serological profile were normal.
PROVISIONAL DIAGNOSIS
Probable APLA syndrome
SAPPORO CRITERIA
 RECOMMENDED TREATMENT

 After 1st thrombotic event-Lifelong warfarin to achieve

INR 2.5-3.5 +/- Aspirin 80mg daily.
 LMWH+Aspirin lifelong to prevent pregnancy morbidity
CURRENT TREATMENT AND FOLLOW-UP

 Warfarin
 Glucocorticoids

Patient received pulse methylprednisolone in the hospital and

her weakness of extra ocular muscle improved, monoparesis
improved partially, but no improvement in vision till date.
THANK YOU