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

BACKGROUND: Infection with tick borne Borrelia Burgdorferi (Lyme disease) can without treatment rarely develop into a chronic phase. Secondary Normal Pressure Hydrocephalus (sNPH) based on chronic infection with Borrelia Burgdorferi (Bb) is an even rarer entity, that with the right treatment is potentially curable. CASE PRESENTATION: A 67-year-old male with a slow onset of progressive balance problems, also presented unspecified dizziness, urge feeling, neck soreness and discrete cognitive complaints. An MRI scan revealed an enlarged ventricular system compatible with NPH. After further liquor dynamic procedures, cerebrospinal fluid (CSF) was analysed with the surprising results of lymphocytic pleocytosis, and signs of increased antibody production. Microbiology revealed chronic neuroborreliosis and the patient was treated with antibiotics accordingly. At the one-year follow-up no symptoms remained and the ventricular system almost normalized. CONCLUSIONS: We describe the 7th published case of sNPH secondary to chronic Borreliosis in a previous healthy adult. Existing published literature has been reviewed and previous cases showed similarly nearly full clinical recovery. Primary/idiopathic NPH (iNPH) is treated with the surgical intervention of ventriculoperitoneal shunt and can be mistaken for a sNPH. The awareness of rare causes of sNPH like chronic Borreliosis is important as it is easily treated non surgically.

Secondary normal pressure hydrocephalus following pituitary apoplexy: A case report.

Background: Although secondary normal pressure hydrocephalus (sNPH) can occur in various central nervous system diseases, there are no reports of sNPH caused by pituitary lesions. Herein, we present a unique case of sNPH caused by pituitary apoplexy. Case Description: A 70-year-old man was transferred to our hospital because of a sudden onset of headache and loss of consciousness. The cerebrospinal fluid (CSF) test showed slightly elevated cell counts and protein levels but a negative CSF culture test. Magnetic resonance imaging showed a dumbbell-like cystic lesion with hemorrhagic change at the sella turcica. From the above, the patient was diagnosed with aseptic meningitis caused by pituitary apoplexy. Pituitary hormone replacement therapy was undertaken, and his symptoms fully improved. However, two months later, he complained of a gait disturbance and incontinence that had gradually appeared. Brain imaging with computed tomography showed no ventricular enlargement compared with initial images, although the lateral ventricles were slightly enlarged. As a CSF drainage test improved his symptoms temporarily, sNPH with possible longstanding overt ventriculomegaly in adults (LOVA) background was suspected. We performed a lumboperitoneal shunt (LPS) placement, which improved his symptoms. Conclusion: This case suggests that sNPH can develop even after a small subarachnoid hemorrhage caused by a pituitary apoplexy in LOVA patients. If the aqueduct of Sylvius is open, sNPH with a LOVA background can be successfully treated with LPS placement.

Secondary Normal-Pressure Hydrocephalus in Rheumatoid Meningitis.

Normal-pressure hydrocephalus (NPH) is a common cause of gait apraxia, cognitive impairment, and urinary incontinence in the elderly. It is usually a primary idiopathic disorder but can be secondary. We present a case of secondary NPH due to biopsy-confirmed rheumatoid meningitis initially refractory to intravenous (IV) immunotherapy. Our patient reported an excellent response right after shunting. Her gait remains normal one and a half years later. We searched PubMed for similar cases of rheumatoid meningitis with gait abnormality for additional clinicopathologic discussion. The patient's movement disorder initially improved with steroid taper. However, she developed progressive symptoms, later on, refractory to IV solumedrol and rituximab. She underwent ventriculoperitoneal shunting (VPS) and reported an outstanding outcome. This is the first reported biopsy-confirmed case of rheumatoid meningitis causing NPH to undergo shunting for immediate improvement. Previous cases of rheumatoid meningitis-associated Parkinsonism have improved with steroid induction. Although our patient's rheumatoid arthritis is now controlled, her case illustrates that NPH in autoinflammatory conditions may not recover with immune suppression alone. VPS is an option for a faster response in secondary NPH due to rheumatoid meningitis or other inflammatory disorders with progressive symptoms despite standard induction therapy.

The Dilemma of Hydrocephalus in Prolonged Disorders of Consciousness.

Prolonged disorders of consciousness (DOC) are considered to be among the most severe outcomes after acquired brain injury. Medical care for these patients is mainly focused on minimizing complications, given that treatment options for patients with unresponsive wakefulness or minimal consciousness remain scarce. The complication rate in patients with DOC is high, both in the acute hospital setting, as in the rehabilitation or long-term care phase. Hydrocephalus is one of these well-known complications and usually develops quickly after acute changes in cerebrospinal fluid (CSF) circulation after different types of brain damage. However, hydrocephalus may also develop with a significant delay, weeks, or even months after the initial injury, reducing the potential for natural recovery of consciousness. In this phase, hydrocephalus is likely to be missed in DOC patients, given that their limited behavioral responsiveness camouflages the classic signs of increased intracranial pressure or secondary normal-pressure hydrocephalus. Moreover, the development of late-onset hydrocephalus may exceed the period of regular outpatient follow-up. Several controversies remain about the diagnosis of clinical hydrocephalus in patients with ventricular enlargement after severe brain injury. In this article, we discuss both the difficulties in diagnosis and dilemmas in the treatment of CSF disorders in patients with prolonged DOC and review evidence from the literature to advance an active surveillance protocol for the detection of this late, but treatable, complication. Moreover, we advocate a low threshold for CSF diversion when hydrocephalus is suspected, even months or years after brain injury.

Characterisation of the complexity of intracranial pressure signals measured from idiopathic and secondary normal pressure hydrocephalus patients.

Hydrocephalus is a condition characterised by enlarged cerebral ventricles, which in turn affects intracranial pressure (ICP); however, the mechanisms regulating ICP are not fully understood. A nonlinear signal processing approach was applied to ICP signals measured during infusion studies from patients with two forms of hydrocephalus, in a bid to compare the differences. This is the first study of its kind. The two forms of hydrocephalus were idiopathic normal pressure hydrocephalus (iNPH) and secondary normal pressure hydrocephalus (SH). Following infusion tests, the Lempel-Ziv (LZ) complexity was calculated from the iNPH and SH ICP signals. The LZ complexity values were averaged for the baseline, infusion, plateau and recovery stages of the tests. It was found that as the ICP increased from basal levels, the LZ complexities decreased, reaching their lowest during the plateau stage. However, the complexities computed from the SH ICP signals decreased to a lesser extent when compared with the iNPH ICP signals. Furthermore, statistically significant differences were found between the plateau and recovery stage complexities when comparing the iNPH and SH results (p = 0.05). This Letter suggests that advanced signal processing of ICP signals with LZ complexity can help characterise different types of hydrocephalus in more detail.