Dr Hussain Azhar

A 13 years old girl Tehmina d/o Mehmood, unmarried, resident of Orangi Town, Karachi, with no known co-morbids, presented in ER with the complaints of:

Fever -------------- 1 MonthLower Limb Weakness -- 3 days

According to the patient, she was alright 1 month back, then she developed high-grade, intermittent fever, with rigors and chills. Fever spike occurred almost daily in the evening. There was no associated cough, urinary problem, shortness of breath, rash, sore-throat, change in bowel habits, severe fatigue, yellowish discoloration of sclera.

She took anti-malarials and anti-pyretics from a doctor, and the fever started to resolve. But she noticed mild to moderate pain in her Left Leg on 1st of September, followed by weakness in her Left Leg, which progressed rapidly to complete loss of power in her Left Leg.

Then she developed mild pain in her Right Leg within 12 hours of Left Leg weakness and had complete weakness in her Right Leg too, followed by urinary retention and constipation

On the day of the weakness, she was taken to a local Doctor, who gave her Inj Mecobalamin, Inj Novidat, and Tab Deltacortil 3 OD

And then referred to CHK

No h/o any confusion or altered consciousness, limb weakness or numbness, double vision, pain in eye, headache, trauma to head or back, difficult in swallowing or speaking, dizziness, tinnitus, gait or balance problem, vomiting, drooping of eyelid, improvement in weakness after rest

No h/o any rash, mouth ulcers, hair loss, arthralgias

Blood Transfusion 2 years back for anemia. According to the patient, she used to feel

weak, and a local GP advised to transfuse blood.

No labs available No h/o jaundice, blood loss, worms infestation,

dietary preference or avoidance No h/o weakness, visual loss, sphincter

disturbance, dizziness, gait disturbance in the past

Menarche not achieved yet

Student of class 7th as well as Madrassa student

Sleep disturbed ( no obvious cause)Appetite reducedNo diet preferences or avoidanceConstipatedFoley’s catheter passed due to

retention

No h/o similar weakness in any family member

Father died of heart trouble 5 years back

Mother and 3 brothers alive and healthy

Next door neighbour had Pulmonary TB and our patient used to visit her frequently

Lower class

Young adolescent girl of average height and lean built, lying comfortably on the bed, conscious and oriented to time, place, and person.

BP = 100 / 60 mmHgPulse = 100 / min RegularRR = 18 / minT = 98.8 degree F

No evidence of anemia, jaundice, cyanosis, clubbing, dehydration, edema

Thyroid not palpableLymph nodes not enlarged JVP not raised

HMF – IntactCN – Intact ( Fundoscopy –

Normal )Motor System in Upper limbs

Bulk , Tone, Power, Reflexes – Intact in Upper limbs

Co-ordination (Finger-Nose) – Intact

• Bulk – Normal• Tone – Reduced in both lower limbs• Power – 0/5 in both Lower Limbs ( hips,

knees, ankles, and toes)• Reflexes – 0 /4 ( even after

reinforcement)• Superficial reflexes – Plantars and

Abdominal reflexes absent• Gait – CNBA• Coordination – CNBA

• Touch• Pain• Temperature• Pressure Intact• Vibration• Joint Position Sense• Proprioception• No Sensory level on the trunk

Signs of Meningeal Irritation – AbsentExamination of the Spine :

No swelling, gibbus, deformity, tuft of hair in lower spine

No tenderness Movements normal without any pain or

limitation

ChestCVSAbdomen UnremarkableHEENTSkin

• A young girl presented with rapid onset of bilateral complete leg weakness with urinary retention and constipation, preceded by 1 month of febrile illness.

• On examination, she is alert, vitally stable. She has 0/5 power in both lower limbs, with hypotonia and areflexia on DTRs and abdominal reflexes, mute plantars, but with intact sensations including perineal sensations. Rest of the examination is unremarkable

Neuropathy ( Motor and Autonomic) / GBS

Cauda Equina/ Conus Medullaris Syndrome

Spinal Cord Anterior Horn Cell Disorder

MyelitisMultiple Scrleosis ( 1st episode)

Hb – 9.8 gm%MCV – 93 flTLC – 8600 / cubic mmPlatelets – 620,000 / cubic mmUCE, LFTs, INR – WNL HBsAg , Anti HCV Ab – Non-ReactiveESR – 44 mm in 1st hourCXR

Asymmetric Sensorimotor Axonal Polyneuropathy more marked on Lower Limbs

Sural Nerves - Unrecordable

Advised to repeat NCS after one week

• Glucose 59 mg%• Protein 41 mg%• TLC 30 / cubic mm• PMNs 10%• Lmphos 90%• RBC 80 / cubic mm• Pus cells Few• Organism Nil

Abnormal signal intensity changes seen in the lower dorsal cord parenchyma, with slight dilatation

Axial images reveal hyper-intense signals in the cord, with the possibility of septations

Thoracic cord meninges are also hyper-intense, suggestive of Arachnoiditis

Tuberculous Myelitis

as Acute Flaccid Paralysis and Sphincter disturbance

with Axonal Polyneuropathy

Retrospective Localization of the Lesion

D/D of Acute Flaccid Paralysis Why not a Simple Polyneuropathy? Cauda Equina vs Conus Medullaris

syndromes Transverse Myelitis Diagnostic Criteria Myelitis with Polyneuropathy Neuropathy with TB

Findings are consistent with LMN lesion in Lower limbs with sparing of the Upper limbs

So it can by any LMN disorder localized to Lower limbs i.e. Anterior Horn Cells of the spinal cord Peripheral Neuropathy NMJ disorder Muscle disorder

Brainstem Stroke , Encephalitis Acute Anterior Poliomyelitis Acute Myelopathy ( Cord SOL / TM) Neuropathy ( GBS, Post-Vaccination,

Diphtheria, Heavy Metals, Vasculitis, Critical Illness)

NMJ disorders ( MG , LES) Muscle Disorders ( Hypokalemia,

Hypophosphatemia, Inflammatory myopathy, Rhabdomyolysis, Periodic Paralyses)Hughes RAC, Comblath DR. GBS. Lancet. 2005: 366: 1653 - 66

According to the standard teaching practices: Following features are NOT a feature of Polyneuropathy: UMN signs Unilateral face, arm, leg signs Prominent Bowel or Bladder disturbance Sensory level Hyperventilation

( Harrison’s Principles of Internal Medicine 17th ed: pg 2653: t379-2 )

Although 5 – 10% of GBS cases have bladder involvement , this is an exception, rather than a rule

Prominent bladder involvement is feature of: Spinal cord problem ( with LMN at the level

of lesion and UMN below the level) Frontal lobe disorder ( with UMN signs in LL

and behavioral, speech, gait disturbances)

Sensory Level ----- Spinal Cord Disorder

No sensory level ---- Spinal Cord disorder

not excluded

Cauda Equina Vs

Conus Medullaris

Conus Medullaris Syndrome

Cauda Equina Syndrome

Presentation Sudden and bilateral

Gradual and unilateral

Reflexes Knee jerks preserved but ankle jerks affected

Both ankle and knee jerks affected

Radicular pain

Less severe More severe

Low back pain

More Less

Conus Medullaris Syndrome

Cauda Equina Syndrome

Sensory symptoms and signs

Numbness tends to be more localized to perianal area; symmetrical and bilateral; sensory dissociation occurs

Numbness tends to be more localized to saddle area; asymmetrical, may be unilateral; no sensory dissociation; loss of sensation in specific dermatomes in lower extremities with numbness and paresthesia; possible numbness in pubic area, including glans penis or clitoris

Motor strength

Typically symmetric, hyperreflexic distal paresis of lower limbs that is less marked; fasciculations may be present

Asymmetric areflexic paraplegia that is more marked; fasciculations rare; atrophy more common

Conus Medullaris Syndrome

Cauda Equina Syndrome

Impotence Frequent Less frequent; erectile dysfunction that includes inability to have erection, inability to maintain erection, lack of sensation in pubic area (including glans penis or clitoris), and inability to ejaculate

Sphincter dysfunction

Urinary retention and atonic anal sphincter cause overflow urinary incontinence and fecal incontinence; tend to present early in course of disease

Urinary retention; tends to present late in course of disease

1. Acute transverse myelitis and acute motor axonal neuropathy developed after vaccinations against seasonal and 2009 A/H1N1 influenza.Sato N, Watanabe K, Ohta K, Tanaka H.

Intern Med. 2011;50(5):503-7. Epub 2011 Mar

2. Simultaneous transverse myelitis and acute motor axonal neuropathy in an adult.Saidha S, Renganathan R, Spillane J, McNamara B, Fanning N, Ryan AM.

J Neurol Neurosurg Psychiatry. 2008 Nov;79(11):1302-3. 

3. Simultaneous central and peripheral nervous system involvement in systemic lupus erythematosus.Ilniczky S, Kamondi A, Arányi Z, Várallyay G, Gaal

B, Szirmai I, Nagy G.Ideggyogy Sz. 2007 Sep 30;60(9-10):398-402.

4. Concomitant transverse myelitis and acute motor axonal neuropathy in an adolescent.Howell KB, Wanigasinghe J, Leventer RJ, Ryan MM.

Pediatr Neurol. 2007 Nov;37(5):378-81.

Such dual lower motor neuron pathology may be associated with a poorer outcome, and indicates simultaneous central and peripheral immune-mediated injury.

There may be a place for combined therapy with immunoglobulin and corticosteroids in patients with transverse myelitis and inflammatory neuropathy.

The causes of peripheral neuropathy associated

with tuberculosis may be:1.Immune-mediated neuropathy2.Direct invasion of nerves3.Granulomas in the peripheral nerve4.Vasculitic neuropathy5.Compressive neuropathy6.Meningitic reaction7.Toxic effects of antituberculous chemotherapy.

J Neurol Neurosurg Psychiatry 2002;73:769–772

Neuropathy occurs as a complication of meningitis, causing spinal nerve root and cranial neuropathies (especially cranial nerves II, III, VI, VII, and VIII).

Cranial nerve involvement may result from longstanding increased intracranial pressure, direct effects of the tuberculous exudates on the nerve, and arteritis of the nerve root, within exudates, has been described.

Isolated tuberculomas in the optic nerve have been reported.

Local pressure in the nasopharynx (cranial nerves IX, X, and XI palsies), in the middle ear and mastoid (cranial nerve VII palsy and hemifacial spasm), and chest (recurrent laryngeal nerve palsy), have all been observed.

Carpal tunnel syndrome may result from tuberculous tenosynovitis in the hand.

The association of tuberculosis with Guillain-Barre syndrome is uncertain, although patients have been reported with both conditions coexisting. This may be a chance association.

INH induced Polyneuropathy: initially sensory, later motor with features of axonal degeneration.

This should be prevented by coadministration of pyridoxine.

Etahmbutol induced Neuropathy: retrobulbar toxic neuropathy reversible distal sensory peripheral

neuropathy, with late motor involvement, and axonal features.

Streptomycin is vestibulotoxic, but does not cause peripheral neuropathy.

Patient has been started on

ATT with HRZE Dexamethsone

Repeat CSF after 10 days( as advised by Prof Dr Yasmeen)

Repeat NCS after 1 week(as advised by NCS department)

Thank You