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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

From: Normal pressure hydrocephalus secondary to Lyme disease, a case report and review of seven reported cases

Year of publication Sex Age (years) Duration of symptoms at admission Symptoms Clinical findings (incl. Tap-test) Radiology Bb IgM CSF Bb IgG CSF Bb IgG CSF/ serum-ratio Pleocytosis (leucocytes, U/mm3) CSF-protein
(mg/dl.)
Differential diagnosis
1996 [12] Female 76 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 testa (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 57 > 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 76 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 83 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 80 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 71 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 75 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 87 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 67 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 = 117o
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
  1. aA positive Tap-test is a removal of CSF by LBP, that gives an effect of the cognition or gait 30 min to 4 h afterward. MMSE Mini Mental Status Examination, MDRS Mattis Dementia Rating Scale, CERAS “ Consortium to Establish a Registry for Alzheimer’s ”, which is a cognitive test. The cella media index (Evans’ index) = ratio of biparietal diameter of skull to maximum external diameter of lateral ventricles at cella media, SPECT Single-Photon emission computed tomografhy, PCR Polymerase chain reaction studies, CMV Cytomegalo virus, VZV Varicella zoster virus, S-ACE Serum angiotensin converting enzyme, LIT Lumbar infusion test