Table 1 Overview of the characteristics, clinical findings and diagnostics of all published cases to date of sNPH in patients with chronic neuroborreliosis, listed in chronological order

1996 [12]

Female

9 months

General weakness.

Progressive gait and memory problems.

Debut of urine incontinence.

Delayed broad-based gait with leftward drift.

Inability to perform tandem gait or stand on one leg.

Reduced attention and memory.

Abnormal behaviour.

MMSE 20/30.

Tap test^a (40 ml.) with no effect. No LIT.

MRI = Dilated ventricles

not matched by an equal increase of the subarachnoid

space.

Patches of subependymal signal abnormalit

Suggesting NPH.

Positive.

Positive.

12.6

98

(82% lymphocytes).

191

CSF for Treponema pallidum hemagglutination (TPHA) = negative.

1999 [13]

Male

> 12 months

Progressive loss of gait function.

Concentration problems.

Slight urine incontinence.

10 kg. weight loss (in 6 months).

Light distal tetra paresis. Hyperreflexia bilateral.

Slow gait with reduced step length and -height.

Moderate dyscalculia.

MMSE and MDRS according to age.

Tap test (30 ml.) with no effect. No LIT.

MRI = Dilated lateral and 3rd

ventricles without cortical

atrophy.

Periventricular changes.

White matter gliosis in basal ganglions, pons and mesencephalon without post contrast enhancement.

Negative.

Positive.

15.2

130

(Lymphocytic overweight).

380

SPECT = only a weak perfusion deficiency left frontal lope compared to the right. Not significant

2003 [14]

Male

6 months.

Progressive cognitive decline, weight loss and increasing falls because of imbalance.

Debut of urine incontinence.

Bilateral ataxia.

Amnesia for recent events.

Spatiotemporal

disorientation. MMSE =15/30

MDRS = 98/144

Tap test (50 ml.) with no effect. No LIT.

MRI = Dilated ventricles.

Suggesting NPH.

Not reported

Positive.

19.7

250

(60% lymphocytes).

3000

Direct examination and cultures for usual bacteria in the blood and CSF = negative. PCR of CSF = negative for CVM, VZV, Epstein Barr, and herpes simplex viruses. Serological tests for syphilis = negative.

2004 [18]

Female

6 months

Weight loss of 5–7 kg.

Urine incontinence.

Gait instability.

Slight diplopia.

Impaired memory and word finding tested via CERAD.

MMSE = 18/30,

Tap test (unknown ml.) with effect. No LIT.

MRI = Enlarged ventricles suspicious for NPH.

Not reported

Positive

Significantly elevated

69

3542

Not described.

2008 [15]

Female

6 months.

Progressive loss of memory and gait problems, now needing support to walk.

Normal bladder control.

Slow, wide

based gait with short shuffling steps. Turning nearly impossible.

Bilateral mild ataxia.

Reduces attention.

Amnesia for recent events

Spatiotemporal disorientation.

MMSE =21/30.

Tap test (50 ml.) = markedly effect. No LIT.

MRI = Dilated ventricles and periventricular lesions

No post contrast enhancement. Suggesting NPH.

Positive.

Positive.

Significantly elevated.

45

(90% lymphocytes).

Elevated.

Not described.

2011 [19]

Female

9 months

Progressive confusion and lability of mood. Memory loss to dependency of daily living.

Weight loss of 15 kg, and daily nausea.

Gait instability with falls.

Debut of urine incontinence (2 weeks).

MMSE = 17/30,

IDSR = 18 (if 7–22, suspicion for Alzheimer’s dementia),

Loss of second language.

Tap test or LIT not reported.

MRI = atrophic. Expansion of the ventricles. Cella media index = 3,4 (abnormal if < 4).

Bilateral symmetric mesial temporal lobe atrophy.

Positive

Positive

7.0

964 (lymphocytic overwight)

2351

SGDS = 8 (mild depression). No effect of 4 months antidepressants. FDG-PET-C. = normal for age. Pupillooccilation = no sign of dementia.

Gastroscopy and coloscopy i.a.

ANA, HCV-Ab., HIV-Ab, Syphillis Ab = negative

2016 [16]

Female

10 months.

Abdominal discomfort, nausea, cognitive decline,

occasional urine incontinence.

Rigor and bradykinesia in the right arm. Unsteady,

broad-based, short-stepped gait with forward flexed trunk posture.

Mildly painful nuchal rigidity,

Low mood.

Not fully oriented to time.

Slowing of speech and movements with word-finding difficulties.

MMSE = 20/30

Tap test or LIT not reported.

MRI = mild periventricular white matter changes and slight widening of the lateral ventricles compared to cerebral sulci with a borderline Evans’ index of 0.34 (normal< 0.3) indicating possible early NPH.

Not reported.

Positive.

Highly positive

Lymphocytic pleocytosis

Elevated.

Not described.

2018 [17]

Male

Not reported.

Urine incontinence and progressive weakness and gait problems.

Bilateral hand tremor, dysdiadokokinesis, dysmetria, not orientated in time and place.

Tap test or LIT not reported.

CT = increase in the size of the third and lateral ventricles suggesting communicating hydrocephalus superimposed on cerebral atrophy secondary to chronic lacunar infarcts.

MRI = ventriculomegaly was more likely due to cerebral atrophy than balanced hydrocephalus.

Positive.

Negative.

Not reported.

Lymphocytic pleocytosis

Not reported.

Not described.

Our study (case from 2018)

Male

6 months.

Progressive balance problems, slight cognitive complaints, muscle soreness, urine urge.

Imbalance when walking on a line.

Left sided ataxia OE and UE.

Hyperreflexia.

Word latency.

MMSE = 28/30, ACE = 89/100 (affected animal fluency and s-word fluency).

Tap test not performed, but improvement in some symptoms after first LP.

No LIT.

MRI = dilated lateral and third ventricles, periventricular hyperintensity but no other pathology, especially no post contrast enhancements.

EI = 0,377, CA = 117^o

Negative.

Positive.

11.7

118

(80% Lymphocytes).

93

CSF cultured for bacteria and fungus = negative.

Tests for both HIV (PCR and DNA), cryptococcus (antibodies), HSV and VZV (PCR and antibodies) and mycobacteria and Tuberculosis (PCR) = negative.

Flowcytometry = sign of reactive response, but not a malignant immune response.

Serology for Syphilis = negative. S-ACE for Sarcoidosis = negative