Case presentation. Frini Karaolidou Florentia Savva Demetra Tourva Trainees in Internal Medicine, General Hospital of Limassol, Cyprus

Transcription

1 Case presentation Frini Karaolidou Florentia Savva Demetra Tourva Trainees in Internal Medicine, General Hospital of Limassol, Cyprus

2 Presenting complaint 45-year-old woman presenting to A&E 3 day history of abdominal pain 2 days urine and stool retention 10 days progressively worsening weakness of lower extremities No fever, vomiting, diarrhoea, cough, headache No recent illness or trauma

3 History Medical history: - IUD (unsuccessful attempt to remove last year) Drug history: - None Allergies: - None Family history: - No relevant family history

4 History Social history: From Vietnam, 5 years in Cyprus Lives in a rural area and works as a housemaid No history of unprotected sexual contact No smoking or alcohol consumption No use of illicit drugs

5 General Observation General appearance: looked uncomfortable, relieved when a urinary catheter was inserted HR:76beats/min BP:110/70mmHg Temperature: 36.7ºC RR:18breaths/min SpO2: 98% on room air

6 Physical Examination - pain at the hypogastrium - palpable bladder resolved after urine catheter was inserted - soft and non-tender on palpation - bowel sounds audible and normal - no hepatosplenomegaly

7 Neurological Examination GCS 15/15, alert and well-oriented Reduced tone and muscle power in both lower limbs (3/5 right, 2/5 left) Achilles reflexes absent bilaterally Patellar reflexes present but reduced Diminished sensation of light touch in both lower limbs, but without clear sensory level Normal muscle tone and power in upper limps, normal cranial nerve examination, no meningeal sings present

8 S1 S2 normal and rhythmical Both lungs clear on auscultation No rashes, lymphadenopathy or signs of arthritis

9 Initial Investigations FBC: Hb 13.4 g/dl, WCC 4.21 x10 9, PLT 163 Biochemistry: urea 33mg/dl, creat 0.53mg/dl, Na+ 137, K+ 3.88, LFTs/CPK normal MSU: nil Pregnancy test: NEGATIVE ABG: normal u/s adbomen: no abnormality detected Chest X-ray: no consolidation or fluid ECG: SR HR 80bpm, no ischaemic

10 Diagnostic thoughts Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome) Myelopathy Compressive Inflammatory Non-inflammatory myelopathy - Vascular - Metabolic - Others

11 Further investigations CT Brain with contrast: normal CT Spine (T9-L4): a small intervertebral disc hernia at the L3-4 level with no signs of spinal cord compression.

12 Further investigations Lumbar puncture: Normal opening pressure WBC 200 cells/µl, 90% lymphocytes RBC 20 cells/µl (no xanthochromia) Gram stain and AFB s: negative Proteins 890 mg/dl Glucose 49 mg/dl (oligoclonal bands, viral PCR, cytology and flow cytometry also requested)

13 Back to our differential diagnosis 1)Ruled out compressive aetiology as no structural abnormality or spinal mass (e.g. tumour, haematoma, fracture, intervertebral disc) 1)Confirmed inflammation

14 Back to our differential diagnosis Could it be Guillain-Barré syndrome? Could it be Acute myelitis?

15 Back to our differential diagnosis Characteristics Acute myelitis Guillain-Barré syndrome Distinguishing features Motor findings Paraparesis or quadriparesis Ascending weakness LE>UE in the early stages Myelopathy: if UE involvement, often as severe as LE; often no UE involvement GBS: there usually is UE involvement and it is less severe than LE involvement early in the disease Sensory findings Usually can diagnose a spinal cord level Ascending sensory loss LE>UE in the early stages Myelopathy: sensory level usually identified; often no arm involvement GBS: no sensory level; usually UE less affected than LE early in the disease Autonomic findings Early loss of bowel and bladder control Autonomic dysfunction of the cardiovascular system Myelopathy: urinary urgency or retention early and prominent; cardiovascular instability only in severe cases higher than T6 spinal level GBS: urinary urgency or retention less common; cardiovascular instability is more common Cranial nerve findings None Extra-ocular muscle palsies or facial weakness GBS: cranial neuropathies are more common than in acute myelopathy CSF Usually, CSF pleocytosis and/or increased IgG index Usually, elevated protein in the absence of CSF pleocytosis CSF pleocytosis and elevated IgG index may be helpful in diagnosing a patient who is suspected of having GBS from acute myelopathy

16 Back to our differential diagnosis Characteristics Acute myelitis Guillain-Barré syndrome Distinguishing features Motor findings Paraparesis or quadriparesis Ascending weakness LE>UE in the early stages Myelopathy: if UE involvement, often as severe as LE; often no UE involvement GBS: there usually is UE involvement and it is less severe than LE involvement early in the disease Sensory findings Usually can diagnose a spinal cord level Ascending sensory loss LE>UE in the early stages Myelopathy: sensory level usually identified; often no arm involvement GBS: no sensory level; usually UE less affected than LE early in the disease Autonomic findings Early loss of bowel and bladder control Autonomic dysfunction of the cardiovascular system Myelopathy: urinary urgency or retention early and prominent; cardiovascular instability only in severe cases higher than T6 spinal level GBS: urinary urgency or retention less common; cardiovascular instability is more common Cranial nerve findings None Extra-ocular muscle palsies or facial weakness GBS: cranial neuropathies are more common than in acute myelopathy CSF Usually, CSF pleocytosis and/or increased IgG index Usually, elevated protein in the absence of CSF pleocytosis CSF pleocytosis and elevated IgG index may be helpful in diagnosing a patient who is suspected of having GBS from acute myelopathy

17 Further imaging MRI Brain: Normal MRI the entire spine with gadolinium: Fusiform lesions extending over several spinal cord segments with and without gadolinium-enhancing signal abnormality => old and recent areas of LETM

18 LETM Longitudinally extensive transverse myelitis (LETM) is a radiological term which refers to complete or incomplete spinal cord dysfunction with a lesion on MRI that extends over three vertebral segments.

19 Transverse Myelitis methylprednisolone 1g daily iv for five days Followed by Prednisone 50mg/day po With good clinical response

20 Causes of Transverse Myelitis Multiple sclerosis/acute disseminated encephalomyelitis (ADEM) Neuromyelitis optica (Devic's disease) Secondary TM (disease-associated) Idiopathic TM (usually as a post-infectious complication)

21 Transverse Myelitis Multiple sclerosis/ Acute disseminated encephalomyelitis (ADEM) BUT -LETM extremely rare in MS/ADEM -No other focii in the brain -Oligoclonal Bands were negative

22 Transverse Myelitis Neuromyelitis optica (Devic's disease) LETM and optic neuritis are characteristic features More commonly seen in Asia and Africa BUT Serum NMO-IgG antibodies (anti-aquaporin-4 IgG): negative No signs of optic neuritis (MRI, Ophthalmological examination)

23 Secondary (disease-associated) TM Infections including but not limited to West Nile virus, herpes viruses, HIV, HTLV-1, Lyme, Mycoplasma, and syphilis. Ankylosing spondylitis Antiphospholipid antibody syndrome Behçet disease Mixed connective tissue disease Rheumatoid arthritis Scleroderma Sjögren syndrome Systemic lupus erythematosus -No sings of arthritis, rash, livedo reticularis, serositis, night sweats, oral ulcers -ESR,CRP, ferritin: normal -ANA, ds-dna, Ro/SSA, La/SSB, Sm, RNP, ANCA, Antiphospholipid antibodies: NEGATIVE

24 Secondary (disease-associated) TM Neurosarcoidosis: Serum ACE: Normal Normal Chest X-ray Normal serum calcium Paraneoplastic syndromes No anaemia, weight loss, fever LDH, ESR, protein electrophoresis: normal HOWEVER a whole-body CT scan was performed

25 HRCT Chest: - multiple paratracheal LN with maximum diameter 29mm, subcarinal LN 19mm, right hilum LN 16mm - normal lung parenchyma CT Neck, Abdomen, Pelvis: normal

26 A video assisted thoracoscopy (VATS) and LN biopsy was arranged SARCOIDOSIS, LYMPHOMA, CA, NMO???

27 CSF FLOW CYTOMETRY T-cell population with increased CD4:CD8 ratio=8.72 SARCOIDOSIS????? Marangoni et al.2006 proposed deleting the Kveim test, serum ACE and chest X-ray from Zajicek criteria and including high resolution chest CT, bronchoalveolar lavage with a CD4:CD8 ratio of greater than 3.5 and a CSF CD4:CD8 ratio of greater than 5

28 Biopsy of 4 enlarged LN: Non-caseating granulomas were seen, indicating sarcoidosis as the most likely underlying diagnosis Our patient has NEUROSARCOIDOSIS!

29 Further Management & progress AZA 5Omg BD in order to taper steroids Physiotherapy Able to walk with a zimmer frame Still has a urinary catheter Improvement can be expected up to 1 year following commencement of treatment

30 Thank you QUESTIONS?