CASE REPORT Daina Pastare Latvia Case report Male, 50 years 2013 - - PowerPoint PPT Presentation

CASE REPORT

Daina Pastare Latvia

Case report

• Male, 50 years
• 2013 – numbness in his left cheek
• 2014 – weakness in right leg, «could not longer play basketball»
• From 2015 – slowly progressing weakness in legs, balance problems
• Arterial hypertension, Tab. Perindopril 10 mg/day

Clinical findings - 2016

• Slightly diminished sensation on left side of the face
• Central type left facial palsy
• Sustained nystagmus on horizontal gaze
• Mild paresis ir right arm – BMRC grade 4
• Reflexes dextra (+++) > sinistra (++), extensor plantar response dextra
• Mild truncal and limb ataxia dextra>sinistra, mild instability in Romberg’s test
• Milda gait ataxia
• Fully ambulatory
• EDSS = 2.5

2016 2018

2016 2018

2018

Laboratory

• Routine blood and urine tests – N
• ANA IgG; ENA IgG; ANCAc IgG (PR-3 IgG); ANCAp IgG (MPO IgG); ds DNS IgG; antiphospholipid

IgM and IgG; RF; ESR; AKE; Beta 2 glycoprotein I IgG IgM, IgA, lupus anticoagulant – N

• Anti - HIV ½; RPR; Anti HCV, HBs Ag, Borrelia burgdorferi IgM and IgG - negative
• Ceruloplasmin; Cu 24h/urine – N
• Vitamin D3 (25-OH) – 7.0 ng/ml (30-70)
• Vitamin B12 – 81.57 pg/ml (191 – 663)
• PSA - N
• Onconeural antibodies – negative
• Aquaporin 4 IgG antibodies – negative
• Anti MOG antibodies - negative

Lumbar puncture

• Cell count - 5/mkl, total protein – 0.41 g/l, lactate -1.81 mmol/l , glu -3.64 mmol/l , IgG – 40.2 mg/l

(10.0-30.0)

• Oligoclonal bands – negative
• HSV1/2 DNA (PCR); VZV DNA (PCR), CMV DNA (PCR); EBV DNS (PCR) – negative
• Borrelia burgdorferi IgG – negative
• TBC-DNA - negative
• Toxoplasma DNA, cryptococcus antigen, anaplasma phagocytophilus DNA (PCR) - negative
• Flow cytometry – no data about lymphoproliferative disease

Other investigations

• CT chest – no abnormalities, no lymphadenopathy
• CT abdomen – cholecystolithiasis
• Gastroscopy – hyperemic mucosa
• Colonoscopy – no pathology

PET/CT

• Two hypermetabolic lesions in left cerebellum and left middle cerebellar peduncle
• No other hypermetabolic lesions/lymph nodes in body

Brain biopsy

• 2017 - from one of the cerebellar lesions
• Histological differential between chronic inflammatory demyelinating and

histyocytic proliferative process

• The morphology was not absolutely typical for either of the disorders
• Infectious process was also suggested to be in the differential
• Some features raising the possibility of Rosay – Dorfman and Erdham-Chester

disease?

• Genetic testing of hystiocytosis – negative

Brain biopsy

Brain biopsy - 2017

Angiography

• CT angiography – left a. subclavia stenosis 90-100% (subocclusion) ,

right a. vertebralis proximal stenosis 80-90%, left a. vertebralis proximal stenosis 80-90%, left ACI proximal stenosis 45-55%, right ACI proximal stenosis 30-40%

• May 2019 – PTA+stent insertion in left a. subclavia
• Tab. Clopidogreli/Aspirini, Tab. Atorvastatini

Treatment

• 2016, 2017, 2018 – Sol. Solu-medrol 1000 mg/ 5 days, no significant

improvement

• 2017 - PLEX (5x) – no significant improvement
• 2017 – intermittent i/v Sol. Cyclophosphamidi - progression
• 2018 – now: Sol. Rituximab (4 infusions) - ongoing progression

Clinical findings - 2019

• Bilateral severe sustained nystagmus
• Central type left facial palsy
• Moderate dysarthria, dysphonia
• Moderate dysphagia
• Episodic hemifacial spasm in left side
• Mild paresis ir right arm – BMRC grade 4
• Both legs - BMRC grade 5
• Reflexes dextra (+++) > sinistra (++), bilateral extensor plantar response
• Moderate bilateral spasticity in legs dextra > sinistra
• Urinary incontinence
• Severe instability in Romberg’s test
• Bilateral moderate limb ataxia dextra>sinistra; legs > arms
• Severe gait ataxia, spastic – atactic gait, bilateral assistance > 5 meters (EDSS=6.5)