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

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

^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