Post

Paraneoplastic Limbic Encephelitis

My dad died from paraneoplastic limbic encephelitis on jan 5ht 2010 after more than 4 months in the hospital and the local hospice.
We had to make the diagnosis ourselves as a family as we knew there was something very seriously wrong and all the tests for strokes,and viruses and infections drew a blank.
Dad had never been ill in his life and had just had his annual fitness check at his gp 2 weeks previous to collapsing with a clonic tonic seizure whilst driving his car.fortunately he had just parked up on my driveway but the engine was still running and he had locked himself in.
My son came to his aid whilst i called  emergency services.My son got into the car by forcing the drivers windown down as it was slightly open.
He slid the drivers seat back to recline position,and commenced primary observations.i climbed into the back seat and held dads head in a safe positon as he continued to fit intermittently,his breathing was laboured,his skin cold and clammy and his face grey.He was making awful gurgling sounds and was unconscious throughout.
After several minutes a paramedic arrived on site via bike..he took control of the situation and asked all relevant questions before placing heart rate monitor and oxygen mask.dads sats had slumped to 83% and his heart rate was irratic and extremely high.
By the time the ambulance arrived dad was starting to come round a bit and though he seemed somewhat confused he managed to get to his feet and into the ambulance but didnt know what all the fuss was about.
Upon arrival at the hospital we sat with him as the doctor questioned him and us as to what had happened,he couldnt recall and it transpired that he had lost 3/4 recent years in time and thought the current prime minister was harold wilson.The docs were concerned and admitted him to the acute admissions for further observation and tests.
Dad was never the same from that day onwards.something had tripped in his brain and caused irreversible damage to memory orientation personality and he was very confused which was totally out of character.
We were convinced he had had some kind of stroke,but his physical capabilities remained unchanged.
Something else was not right,he had forgotten he was a smoker for the majority of time,even though he had smiked for around 55 years.
Occasionally he would remember and start rumaging in his top shirt pocket and then walk around the room as though looking for something,when we asked what he was looking for he would say,that stuff that was in my pocket.
we quickly learnt to avoid the cigarette issue by handing him his keys or mobile phone.Which he failed to recognise,
Days of tests turned into weeks and weeks and diagnosis went from stroke to epilepsy to dimentia,infection and so on...
No one seemed to know what to do with his or how to deal with his increasingly erratic and unusual presentation.
We took to sitting by his bed  from morning til evening as this was proving to be the only way of keeping him pacified.
The ward sister rang me at home on several occasions to ask if we could come in and sit with him as he had taken to wondering off at random,into other wards,and departments.
He picked up repeated hospital acquired infections and also suffered numerous bouts of aspirated pneumonia and his health fell into a rapid spiral downwards.
He had all the medics flummoxed over a definitive diagnosis as non of the symptoms were consistent other than they were neurological,and stemmed from the hippo campus region of the brain.
His blood pressure would have peeks and troughs,he was labelled diabetic for a while as his levels fluctuated wildly,his abdomen would swell as he had raging thirsts and would down 2/3 jugs water which had to be restricted many times.
he would hallucinate,seeing things on the wall and people that were not really there.He thought he was in the army barracks where he had trained as a teenager.
As we frantically scoured the internet to look for a condition which reflected dads symptoms we stumbled upon the Encephelitis site and in particular paraneoplastic limbic encephelitis.
Bingo,it all fitted into place,now all we had to do was convince to docs.
We were surprised by the reaction when we suggested it to the chest doctor,she said it is an extremely rare condidion in fact so rare that she had never come across a patient with it yet.Nothing more was said on the subject.
Dad suffered series of clonic tonic seizures and after each one he would be back to square one with memory and confusion,then for a few days he would sort of come back to us and even begin to ask where he was and what was wrong with him.
The one night after we had left him for the night after tucking him into bed with the tv on,he suffered a massive fit and this time his heart rate was soaring to such a degree the staff called me in as an emergency and said they had the intensive care team working on him and they were preparing to sedate him and place him on a ventilator as they feared he was about to go into cardiac arrest.They wanted to give his body a rest whilst they continued to search for a diagnosis.He spent the next week in ICU during which time they attempted to conduct a bronchoscopy as they said an xray had showed up a mass on his right centre chest.
The bronchoscopy was inconclusive and after he was sent back to the ward a 2nd bronchoscopy was conducted which again proved inconclusive.
Epilepsy drugs were no longer controlling the seizures and dad continued to aspirate and then become infected,he now also had indicators of mrsa and had contracted cellulosis.
There was a lack of communication between the multi disciplinary team and as a result dad went for a period of 5 days with absoloutely no food or water for fear of him aspirating,his swallowing capabilities had now diminished and he could no longer take oral meds.
He was left for days without basic hygiene care as his behaviour became aggressive at times and his balance and co ordibnation went..
It was horrific beyond belief.I pleaded with the neurologist to reconsider the stroke theory as a we didnt think this was causing dads problems.
She gave in somewhat and after listening to us she altered dads medication  to include kepra and dexamethzone.
The docs had a meeting and finally discussed the possibility of an underlying tumour.a mediastenoscopy was recommended and dad was sent to hull for what was meant to be an overnight stay for  the diagnostic procedure.
The team at hull insisted dad had to be contained in a single room as he showed indicators of mrsa.......dad continued to decline and became les and less chatty,he had several fits whilst at hull and the procedure was delayed for 4 days as docs said dads consent was in question  and they hadnt been informed of his mental state by york hospital.I countersigned the papers and the procedure went ahead despite dad barricading himself in his room and refusing anyone entry until a very patient surgeon managed to talk him round and held his hand down to the theatre.
When a small cell lung carcinoma was first suspected we were informed that if it did prove to be the case dad would be offered chemo and radiotherapy.But in reality they had decided that the tumour was fast growing and too widespread and given dads mental state and other cognitive problems he wouldnt be weel enough to have any form of treatment now.
We never understood this,his final scan hadnt shown any metastates to the brain to account for his symptoms and yet they were still refusing to consider this may be limbic encephelitis.
There were no written up details confirming a sclc on dads notes form hull and yet on his death certificate it states sclc as cause of death.
I objected to this but was told that if i continued to object they would be forced to perform an autopsy on dad,i didnt want this as he had been poked about enough.
I was convinced that my dads medical records proved that he did in fact die form paraneoplastic limbic encephelitis and that he did have a sclc which was small and local at the time of presentation and probably treatable had they followed up the shadow on his lung at A&E 3 weeks prior to being admitted to hospital with some kind of throat spasm.He was sent home with a pack of piriton tablets and told it was probably an allergy.
After making a formal complaint to York hospital and after several meetings with various staff,doctors and nurses they have finally agreed with me that in all likelihood dad did indeed die from PLE and his presentation at A&E was first indication.They also went on to say that neurologists involved could have made more vigorous efforts to look for alternative causes for dads symptoms.
It is unlikely that any amount of intervention would have saved dad and it is unclear as to whether high dose steroids etc would have had any effect on the neurological symptoms.They also said that most cases of sclc are very fast growing and are found too late to treat.
I just find it difficult to accept that a tumour on the lung is deemed unrecognisable on an xray in Sept but then it is widespread and massive and untreatable by Nov even though it hadnt metastasised outside the chest at that point.
I will continue to fight for the death certificate to reflect the PLE........
The more public records show this condition the more may be understood about its effects



pandaface pandaface 46-50 1 Response Jul 15, 2010

Your Response

Cancel

I am so sorry for your loss. I just lost my 69 year old father nearly identically 3 weeks ago. Also a smoker of 50 years, He presented in the fall of 2010 with night sweats, depression, personality changes, rages, narcolepsy, bells palsy causing one side of his mouth to droop and cataplexy (he was STARVING and slim most of life, gained 40 lbs in a month) Finally the swallowing difficulty caused aspiration pneumonia and he nearly died. Then came the hospital battles, the bronchoscophy where they blamed him for being aggressive, so even though they claimed they got a cancer free sample, they flat out lied. Then another biopsy confirmed sclc. Our saving grace was a neurologist named Dr. Oger who diagnosed him properly, fought for my dad with IviG, Steroids The doctors at VGH had their heads in the sand, and were it not for Google, a paraneoplastic brief by a man named Dr. Dalmau and sheer determination on the part of my mum,combatting their misdiagnoses at every turn, my dad wouldn't have had the 10 months we had with him. (My dad had excessive hunger as opposed to thirst, and because the encephalitis causes swallowing difficulty he also spent a week clearly lucid yet starving and given no food or water for a week. And 'blamed' by medical personnel as being 'aggressive and difficult'. The doctors blew off dads case with a diagnoses of lymphoma then lung cancer, refused to treat the cancer with radiation because he 'wouldnt stay still' Are you effing kidding me. (he died with it as stage 1, didn't even even metastasize) and sent him home to die. Thankfully we met with an amazing neurologist named Dr. Oger who LISTENED, confirmed Anti-MA2 encephalitis and began treatment with iVig antiimmune therapy, steroids for the brain inflammation a few other medications and a host of natural treatments like mistletoe, and other vitamins my mother found through an ortho oncologist. He eventually died from MRSA pneumonia as your dad did. The moral of the story is that if caught early enough people DO survive this illness. Doctors need to be educated about symptoms, or be taught how to use a bloody search engine. Encephalitis Global www.encephalitisglobal.org was also incredibly helpful, so we have asked people to donate in my dads name. For any who come across this post, here are some things to look out for if you are searching for answers:

* Paraneoplastic syndromes (encephalitis as a result) can be caused by an underlying cancer that isn't even big enough to be found yet. The body attacks its own immune system causing brain inflammation (encephalitis)

* The anti-Ma2 syndrome caused by an underlying lung cancer or testicular cancer and is most often seen in males ages 65-75, smokers, diabetes

*Many patients present with:

Dementia or stroke like symptoms

Swallowing difficulties from the brain inflammation leading to aspiration of food and hospitalization

One closed eye

Droop in one side of the face (bells palsy)

Fatigue and narcolepsy

Unsteady gait

Cataplexy or weird odd behavior where they are STARVING or thirsty and no amount will quench it

---) No matter how many roadblocks you face, be an advocate for your loved one. Bring print outs of Dr Dalmau's research. Insist on MRIs and CT scans and a lumbar puncture. Insist on blood work, and all screening for lung and testicular cancers

---) Insist on a referral to neurologist who knows about paraneoplastic syndromes and knows to treat with iVig, steroids, and cyclophosphamide

* push for radiation and chemo if at all possible. Eliminating the cancer can often diminish/defeat the encephalitis.

*Don't lose hope

**I am not a medical professional, this is a personal account of my family's story, what happened, what worked and didn't work for us.**



Some sort of pamplet should be made available to every GP, Neurologist and Oncologist, particularly to those with older male patients showing these symptoms and how to prescreen for paraneoplastic syndromes. The more awareness, the less fathers that will succumb to this dreadful illness..



SM