There has been much speculation since actor Robin Williams died regarding the circumstances that led to his demise. Many wonder whether his neurological disease Lewy Body Dementia (LBD) diagnosed only after autopsy triggered his suicide. One will never know for certain what drove this brilliantly talented man to the edge of desperation putting an end to his own life.
However, because of the tragic loss of such beloved celebrity who initially had been given a diagnosis of PD while living only to confirm another less common disease LBD after his death, the question still lingers in everyone’s mind could they or their loved ones be afflicted with such disease and not know it?- such a strange word for so many…
Thus, I would like to discuss the topic of dementia in its various forms and its relation to Parkinson’s disease.
First, I would like to put everything in perspective- dementia is defined as loss of previously acquired cognitive skills including language and complex motor skills, of which Alzheimer’s is by far the most common type affecting well over 5 million American or about 1/9 patients 65 or older.
Dementia is then subdivided in to cortical (pertaining to higher-cerebral- cortex and cognitive function such as memory and language) and subcortical (involving the structures ‘underneath’ the cerebral cortex –i.e. the connections between the different lobes). Subcortical dementia is a clinical syndrome characterized by mental slowness, depression, apathy, impaired cognition and forgetfulness.
Unlike Alzheimer’s (cortical dementia) where there is an actual loss of neurons- in Parkinson’s dementia since it’s a subcortical dementia, the neurons are preserved only the chemicals are diminished, and the wiring is faulty making retrieval cumbersome and slow. In Alzheimer’s, as in other forms of cortical dementia, the information once lost is gone- reason why giving cues does not help to remember as it does for those suffering subcortical dementia where Parkinson’s dementia is the prototype. Therefore, in subcortical dementias like seen in Parkinson’s the possibility exists of being able to create new pathways between the various structures of the brain thus potentially thwarting the progression nor severity of disease. this is why it is EXTREMELY important to obtain early diagnosis by a neurologists because although there is no cure for dementia of any type subcortical ones can be slowed down significantly or halted if correct diagnosis is given and treatment started early. One of the biggest therapeutic advantages to a better prognosis and quality of life is the institution of non-traditional modalities such as exercise and art therapy in addition to traditional treatments.
Some neurologists / movement disorder specialist including myself believe there is a spectrum of disease in which you have Alzheimer’s on one end of the spectrum with Parkinson’s at the other end …with about 2 million people. Then you can have as many diseases as you can think of with various combinations ..including all the Parkinson’s plus syndromes (MSA, PSP, etc. closer to PD) & dementia syndromes like Fronto-temporal dementia, pick’s disease, etc.
Lewy body dementia lies at the crux of the see-saw smack down the middle. Then there are those rare patients who also truly have BOTH Parkinson’s and Alzheimer’s but those are even more rare. The reality is that many patients have mixed symptoms most frequently due to vascular disease. This is why it is imperative to ALWAYS have a brain scan at onset of diagnosis or if things don’t match up. More PD patients are in fact more likely to have a variation of Parkinson’s and vascular dementia then Alzheimer’s or other PD Syndromes. This is because most Alzheimer patients are typically otherwise extremely healthy and have no other risk factors while (thus usually look normal in appearance and mannerism at presentation) those with Parkinson’s disease can have and usually do have other illnesses including risks for stroke ( which I believe is greatly enhanced by medication effect especially in woman as a study of PD women showed higher incidence of stroke compared to men- most likely due to uncontrolled hypertension caused by dopamine and dopamine agonists).
So how do you diagnose?-
First, we must remember that of all the dementias, Alzheimer’s is by far the most common followed by vascular dementia caused by strokes. Of course by far Parkinson’s is the more common of the movement disorders second only to essential tremors. After understanding of this knowledge, it is both a matter of recognition of patterns (comes only through extensive training and years of seeing patients in a particular field- hence need for neurologists/MDS) and a numbers game in diagnosing- meaning that common things happen commonly. Yet, a GOOD NEUROLOGISTS ALWAYS HAS THE RARE DISEASES IN THE BACK OF THEIR MIND when things are not presenting, progressing or responding as they should!
Second, listening to the patient and taking a good history is key which means that you as a patient or caregiver MUST try to give as concise and detailed account as possible of symptoms including timeline –
- what came first?
- how long and far between onset of other symptoms?
- are symptoms rapidly progressing?
- are they progressing in a step like manner – meaning worsening then plateauing then declining again?
Third, equally important, especially when symptoms are very early and unclear, is to have continuity of care by same doctor for several months until picture is cleared- sometimes unfortunately we become impatient and want to know what is wrong with us so desperately that we jump from doctor to doctor giving them only a glimpse into the real pathology. Thus, each doctor only sees just one moment in time instead of the whole picture making diagnosis more difficult for any one person until it becomes so obvious. However, by the time it is easy to diagnose even by none experts frequently so much time has been lost that treatments may be ineffective due to advancing disease ultimately robbing us of not only our peace of mind but also diminishing our quality of life.
Characteristics of common dementias with/without Parkinson’s:
Alzheimer’s disease is loss of speech& language, along with memory loss. Immediate or recent memory is impaired while remote memory is preserved. Patient’s usually get lost in familiar places, do not recognize familiar faces, experience loss of previously acquired skills- complex motor skills. Initial presentation includes loss of smell, irritability, depression, personality changes, and apathy. Withdrawal is a common presenting characteristics along with fender benders – these patients ARE NOT hallucinating when they first present. If diagnosed earl, they respond well to acetylcholinesterase inhibitor (e.g. Aricept, Razadyne, Exelon) and Namenda (Memantine) which can not only improve quality of life but delay hospitalization into a facility. It is a chronic progressive disease which occurs over 20-30 years. The incidence increases with age- although not typically hereditary there are two Alzheimer’s genes which are familial Presenilin 1 & 2. Risks of developing ALZHEIMER’S are illiteracy, low education, low socioeconomics, and lack of exercise, high blood pressure and diabetes.
Lewy body dementia – main characteristic is hallucinations (e.g. auditory, olfactory, visual, tactile, and gustatory) at the onset of disease, along with vivid dreaming, severe REM behavior, and early visuospatial impairment in absence of memory loss along with stiffness, slowness, marked bowel, & bladder problems. The key is that introduction to dopamine agonists and dopamine exacerbates or brings to the forefront the hallucinations. Also Namenda usually given for dementia /Alzheimer’s will worsen symptoms of hallucination, becoming psychotic delusional and extremely agitated. However, other memory medicines can improve quality of life. Incidence of LBD is. 21/100,000. This type of illness is a rapidly progressive disease 5-8 years. More common in men 4 to 1 usually in their 70’s.
Fronto-Temporal dementia a.k.a. Pick’s disease– they have significant behavioral and personality changes, interpersonal relationships, including language disturbances and alterations in muscle/motor function. They are caused by disorders involving the protein called TDP43 or the tau protein– why the lobe frontal lobe no one really knows. They usually occur in the 50’s and 60’s however some people may develop as early as their 20’s or as late as their 80’s. there is a behavior variant and a language variant. both the behavioral variant and the language variant are much less common than Alzheimer’s disease in those over 65 years of age. However, in the 45-65 year range both of these are as common as young onset Alzheimer’s. Currently it is estimated that around 60,000 people have FTD the majority of whom are in the young age group. the thing that will distinguish Alzheimer’s and FTD is progression and genetic abnormalities. However,what is interesting is the fact that within the realm of FTD’s we have PSP (Progressive Supranuclear Palsy) and CBGD.
- CBGD (corticobasal ganglionic degeneration) presentation includes more personality and behavior changes at the beginning of disease like FTD’s, even in absence of memory loss. Patients have trouble producing and comprehending language. Both of these have a quick rapid progression. FTD is closer to Alzheimer’s so no PD symptoms unlike CBGD which commonly is unilateral (even when it becomes bilateral there is an obvious dominance); has alien hand. Typical duration of CBGD is 6 years. Patients with CBGD usually are between 50- 70 years of age and it comprises about 1% of population. Tremors, rigidity, muscle spasm, involuntary eyelid spasm, sensory loss, significant swallowing difficulty are also part of the presentation and clinical picture. There is no good treatment for these patients; they poor response to both Alzheimer’s drugs and to PD meds because they have both cortical and subcortical dementia. The best treatment especially early on is speech and physical therapy ; when started early in disease process it can significantly improve quality of life particularly since dysphasia and dysarthria (slurred speech) are early signs leading to frequent aspiration and aspiration pneumonias. These patients also have significant apraxia – this is the inability to perform purposeful movements especially when asked such as making an “okay” sign with your hands.
- PSP-a.k.a.- Steele-Richardson-Olswenski syndrome- progressive supranuclear palsy-initial symptom in 2/3 of cases is loss of balance , difficulty walking-fast walking bumping into objects or people, and changes in posture- lunging forward when mobilizing. the other common early signs include general slowing of movement, change in personality and visual symptoms due to restricted eye movements especially in the vertical plane-because they can look down very well people fall frequently because cant see especially when stepping off a curb or are increasingly messy when eating because can’t see the food. Later dementia develops affecting loss of inhibition and difficulty organizing information. They also have muscle stiffness, swallowing difficulty (usually cause of demise), slurring of speech. Men and women affected equally, about 6/100,000. A poor response to dopamine along with symmetrical onset is the big CLUE along with abnormal eye function which includes eyelid apraxia.
Parkinson’s dementia -early presentation is classic Parkinson’s symptoms which include tremors, slowness, stiffness, and gait impairment; only after 10 plus years do patients get dementia of PD which occurs in up to 50% of individuals. – These patients respond well to PD meds and to acetylcholinesterase inhibitors as well as to Namenda. Exelon works great as do combination of Aricept and Namenda extended release or short acting (now in single formulary called Namzaric). Treatment at early signs is key preceded by adjustment of dopamine levels because the brain is also a muscle and just like the muscles get stiff and slow due to lack of dopamine so does the brain resulting in slow retrieval and weak connections- sometimes all it needs is an extra kick dose of dopamine. Presentation is usually depression, apathy and forgetfulness which is remedied by giving cues. Hallucinations do not occur until late stage and typically are visual. Most visual hallucinations in advance stages are usually benign. A common theme is that of children which typically do not require medication.
Recommended treatment for PD dementia with antipsychotics like Seroquel or Clozaril only if hallucinations are frightening or interfering with care or activities of daily living but first recommend adjusting PD doses then adding acetyl cholinesterase inhibitor if not better then antipsychotic as last result. I recommend that if there is question of memory problems talk to your doctor ASAP and obtain a neuropsych evaluation if necessary which will point to type of dementia cortical/subcortical or both? If have any memory problems or problems with speech, language, gait, coordination, tremors, stiffness or slowness seek first attention of a neurologist who can assess whether a movement disorder specialist is needed.
In summary, knowing the facts will aid in early detection and treatment. In order to achieve this we must be proactive and practice self advocacy because after -all no one knows your capabilities better than you or your spouse/partner so don’t delay seeking medical attention from a NEUROLOGIST if you or loved one have any of these symptoms or have family history of cognitive problems. Plus, do not forget to exercise at least 15 minute walk 3x a day because can significantly decrease risk of Alzheimer’s dementia especially in women. It may also possibly improve or decrease risk of those with subcortical dementia’s like PD involving basal ganglia by increasing blood and oxygen perfusion to this area. Also because the brain is a muscle we must remember that if we don’t use it atrophies. Therefore, the more you stimulate it and challenge it the more connections it will develop and the lower the risk for getting dementias of any type.
So as we approach anew year make a resolution to Go ahead learn a new language, travel more, take up a new hobby, play with your grandkids, and socialize with your friends outside of social media. These things will not cure our illnesses or prevent us from getting them in the first place but can greatly shift the balance to our favor by decreasing risk of becoming severely cognitively impaired.