Rapidly Progressive Cognitive Impairment Without Delirium

Diagnostic Challenges from the Memory Clinic

Dr Andrew Tarbuck (FRCPsych)Dr Yasir Hameed (MRCPsych)Norfolk and Suffolk NHS Foundation TrustNorwich, UK

Objectives

Describe two clinical cases

Rapidly progressive cognitive decline without delirium

Consider differential diagnoses

Lessons to be learned

The problems with current pathways

Case 1: Rapid Descent

Referred urgently by GP in February 2014

63 years old lady lives with husband

Increasing “confusion”

History of a fall 10 days ago.

Evidence of psychosocial stressors.

GP Referral

Reasons for Referral

New onset of panic attacks with anxiety symptoms (hyperventilation) and perceptual distortions (feeling that the room/walls/people closing on her)

Headaches and burning sensation over her body

Unusual experiences: auditory hallucinations, misidentification of family, rooms and furniture

Disoriented in her own home (unable to work out how to get out through a door)

Background HistoryPast Psych Hx: Overdose in 1970s in context of

relationship breakdown. None since.

Past Med Hx: ME since 1987 and IBS. On Amitriptyline 25 mg nocte for years.

Family Hx: Brother died from complications of alcohol dependence. Daughter under Ix by neurologist for ? Peripheral neuropathy.

Personal Hx: Lives with husband. Daughter lives nearby. Son works as a paramedic.

Initial Assessment

Triaged by Single Point of Access to Working Age Adult Services.

Brief period of input from Crisis Resolution and Home Treatment Team (CRHT)

Speech incoherent and vague

Sleeping 18 hours a day

Unsteady on her feet, rapid decline in mobility and self care

Fluctuating presentation

No fever.

No alcohol or drug misuse.

Initial Assessment (cont’d)

Conscious, but disoriented to person, place and time.

Cranial nerves and peripheral nerves intact. GP COG 3/9. She was able to draw a clock and put hands on.

Her appetite fine (she eats everything we put in front of her)

Husband is unable to cope. Her son and daughter visiting regularly.

Over next few weeksCT scan 2.3.14 and bloods: NAD.

CRHT referred to Dementia Intensive Support Team (nurse-led service). Mirtazapine started. Amitriptyline stopped.

Consultant old age psychiatrist review requested

Findings

Thin, very ataxic & unstable, unable to sit upright, choreoathetoid movements, myoclonic jerks, intermittent hyperventilation.

Speech very slow, halting, word-finding problems, perseveration, logoclonia

Labile mood

No psychotic features

Findings (cont’d)

Very poor concentration, unable to give age, DOB, home address, disorientated in time

Exaggerated startle response, pout reflex

What is your differential diagnosis?

Action

Urgent Neurology referral

Neurology Admission 24.3.15

MRI 26.3.14: Extensive bilateral asymmetrical cortical restricted diffusion with mild associated high signal on T2 and FLAIR images. Sparing of subcortical regions.

EEG: diffusely low, nonspecific changes suggestive of neurodegenerative disease.

MRI

EEG

Outcome

Patient passed away on 21.4.14.

Diagnosis:

Sporadic Creutzfeldt–Jakob disease (CJD)

Creutzfeldt and Jakob

Diagnosis of Sporadic CJD

Other types of CJDVariant CJD: First reported in 1996 in UK. Transmission of

BSE infection from Cattle to human. Compared to sCJD: Age onset is younger, longer duration of illness (1 year or more), initial presentation is psychiatric then neurological. Tonsillar biopsy is used to show abnormal protein.

Familial (Genetic) CJD: Caused by an inherited abnormal gene (a mutation in the human prion protein gene, PRNP). Positive family history and genetic testing can confirm the diagnosis.

Case 2: Bewilderment

79 years old man

Referred in April 2014 with 4 weeks history of interrupted sleep, tearful.

Anxious ++ regarding planned prostate surgery

Bewildered with poor memory

Frontal headache

GP referral

GP COG 1/9 (scored 9/9 in November 2013)

GP started citalopram, he didn’t take it.

No obvious neurological or physical sign

Past Med Hx of bladder stones, BPH, AAA surveillance, AF and cardiac stent. On warfarin.

Psychiatric assessment He had severe ear infection and UTI in March. Rapid deterioration from

this point

Reports of visual hallucinations. Right sided inattention (bumping into walls and doorways) with right left disorientation.

Unsteady on his legs

Episodes of urinary incontinence

Reduced motivation and poor self care. Labile mood.

Word finding difficulty and using wrong words (says “water” when he want to go to the toilet). Non fluent dysphasia.

Cognitive assessment: disoriented, severe cognitive impairment.

Nominal dysphasia (unable even to name pen or watch).

Poor comprehension.

Unable to score any points on formal cognitive testing.

Any suggestions for diagnosis?

Differential diagnosis

CVA with subsequent progression (possibly bleeding)

Intracerebral abscess resulting from his recent ear infection

Subdural haematoma (although no history of recent falls or head injuries)

Some other space occupying lesion

MRI 06 May 2014Solitary 4.2 x 3.4 cm centrally heterogeneous mass in the left pareito-occipital region.

Local mass effect with effacement of the left posterior horn of the lateral ventricle.

Outcome

Patient passed away on 26.07.2014

Diagnosis:

Glioblastoma of left parieto-occipital and to a lesser extent, temporal lobe.

Glioblastoma

The most common and most aggressive type of primary brain cancer.

Current treatment include maximal surgical resection followed by concurrent radio and chemotherapy.

The prognosis is poor, with median survival of about14 months.

Challenges in assessments of rapid cognitive impairment Limitations of the Single Point of Access and

“ageless” service model.

Difficulties in the referral pathways between psychiatry and neurology.

Who should do the initial assessment? (Nurses vs Psychiatrists vs Neurologists)

The rapidly changing clinical presentation.

Take home messages

Very rapid change in mental state with focal neurological features is strongly suggestive of an organic/neurological problems.

Have a high index of suspension for the unusual/rare disorders that present with cognitive impairment.

Joint and liaison work with primary care, psychiatry, psychology and neurology is need to optimise assessment and management.