sarcoidosis is a very rare disease in korea with an incidence of 59 cases in 1998 ( 0.125 cases/100000 people)11 ) .
hydrocephalus is a rare clinical feature with a prevalence of 6% among patients with neurosarcoidosis , as reported by a prospective study2 ) .
secondary hydrocephalus following meningitis , ventriculitis , or hemorrhage may be complicated by septum formation in the ventricular system2 ) .
benzagmout et al.14 ) reported that after the treatment of ventricular shunt and corticosteroid therapy , acute hydrocephalus by neurosarcoidosis was resolved .
we report the case of a 31-year - old man who developed multi - septated hydrocephalus from neurosarcoidosis and was treated with multiple shunt placements .
a 31-year - old man complained of a mild headache in the frontal area without nausea or vomiting for 7 days . on admission , he was confused and disoriented with time and space .
the patient had no fever , and his complete blood count , serum biochemistry profiles and autoimmune antibody screening results were normal .
cerebrospinal fluid ( csf ) analysis revealed leukocytosis ( 53 leukocytes / mm ) with a mononuclear preponderance and markedly increased protein to 2.64
chest radiograph and chest ct scan showed bilateral symmetric hilar and mediastinal lymphadenopathy suggesting sarcoidosis .
gadolinium - enhanced magnetic resonance image ( mri ) of brain revealed hydrocephalus and enhanced lesions in septum pellucidum , infundibulum , aqueduct , corpus callosum , and leptomeninges ( fig .
we performed neuroendoscopic biopsy of the enhanced lesions in the septum pellucidum through the standard right precoronal approach .
histopathological results showed non - caseating granuloma and biopsy specimens were all negative for bacteria , fungi and acid - fast bacilli , which were consistent with neurosarcoidosis ( fig .
ventriculo - peritoneal shunting ( strata valve ; medtronic , ca , usa ) through the right parieto - occipital approach was performed , and intravenous methyl prednisolone was then started at 20 mg / kg / day for 3 consecutive days ; this was followed by prednisolone ( 1 mg / kg / day ) for 4 weeks . on post - operative day 5 ,
the patient showed progressive improvement of confusion and disorientation , and at 3 weeks post - operation , he was discharged without any neurological deficit .
four months after discharge , the patient was re - admitted due to headache , and gait disturbance .
brain ct revealed an enlarged left lateral ventricle with periventricular edema and a collapsed right ventricle , at which a ventricular catheter was inserted ( fig .
gait disturbance including headache improved gradually , and in post - operative week 4 , he was discharged without any neurological deficit .
six months after second shunt operation , he complained of upward gaze palsy , and gait disturbance .
brain ct showed an increased fourth ventricle with periventricular edema and both lateral ventricles were well - collapsed with ventricular catheter in situ ( fig .
gadolinium - enhanced mri of the brain revealed an enlarged fourth ventricle with other enhanced lesions in the left occipital lobe , left cerebellar peduncle ( fig .
therefore , we planned ventriculo - peritoneal shunting for the isolated fourth ventricle by neuronavigation with intravenous methyl prednisolone .
neuronavigator - guided fourth ventriculo - peritoneal shunting was performed via the left lateral transcerebellar approach with neuronavigation ( in2vision ; cybermed , seoul , korea ) .
the csf profile collected at the operative field revealed severe leukocytosis ( 121 leukocytes / mm ) and an increase in the level of protein to 2.03 g / l .
after 2 weeks of steroid treatment , csf collected at the valve dome was normalized ( 6 leukocytes / mm and 0.62 g / l protein ) . on post - operative day 10 ,
upward gaze palsy and gait disturbance gradually disappeared , and the patient was discharged 4 weeks post - operatively with mild neurological deficit .
four months later , the patient 's previous upward gaze palsy and gait disturbance was resolved and ct at follow - up showed a reduction in the size of the fourth ventricle compared to the previous pre - operative ct scan ( fig .
sarcoidosis is a very rare granulomatous disease in korea , and its cause remains unknown .
neurosarcoidosis most often presents with cranial nerve palsies , predominantly affecting the optic and facial nerves , reflecting its predilection for the base of the brain .
the diagnosis of neurosarcoidosis is confirmed by biopsy showing noncaseating granulomas in the absence of organisms or other potential causes . in patients with hydrocephalus
, the treatment depends upon the clinical status . in the case of asymptomatic hydrocephalus , treatment is not required4 ) .
secondary hydrocephalus following meningitis may be complicated by septum formation in the ventricular system , resulting in complex manifestation of csf circulation disturbance7 ) . even though the prognosis for neurosarcoidosis itself is good
, hydrocephalus associated with neurosarcoidosis has poor long - term prognosis with a mortality rate of 75%1 ) .
benzagmout et al.3 ) reported that after the treatment with ventricular shunt and corticosteroid therapy , acute hydrocephalus associated with neurosarcoidosis was resolved . in the present case , we first performed ventriculo - peritoneal shunt in the right lateral ventricle for hydrocephalus in neurosarcoidosis , and observed the slit - like right lateral ventricle 4 months after discharge .
hamada et al.8 ) reported that endoscopic treatment was effective for the treatment of obstructive ventricles in neurosarcoidosis .
further , they suggested that loculated hydrocephalus , which was often treated by placement of an additional shunt , can be better treated with neuroendoscopy since multiple shunt placement was complicated and often results in shunt malfunction8 ) .
it was necessary to perform endoscopic plasty of the occluded right foramen of monro and fenestration of the septum pellucidum for the treatment of hydrocephalus during endoscopic biopsy .
isolated fourth ventricle requires neurosurgical intervention , because impairment of brain stem functions may lead to sudden death12,13 ) .
shin et al.14 ) reported that in the presence of a functioning shunt in the lateral ventricle , treatment options for the isolated fourth ventricle include the placement of an additional fourth ventricular catheter , via a variety of trajectories or endoscopic approaches to open the cerebral aqueduct .
an endoscopic approach can only be performed if a membranous occlusion of the aqueduct is present on preoperative mri .
further , the standard precoronal approach can be used only in cases with a dilated supratentorial ventricular system5 ) . in the present case , because the supratentorial ventricular system was markedly collapsed and both the inlet and the outlet of the fourth ventricle were obstructed by enhanced lesions , the endoscopic procedure was not considered as an appropriate treatment option .
therefore , to minimize the complications related to fourth ventricular shunt insertion , we performed ventriculo - peritoneal shunting via the left lateral transcerebellar approach using a neuronavigator .
the risks of this procedure included brain stem penetration by the ventricular catheter , with focal neurological deficit because of the compressed cerebellum and brain stem expansion by decompressing the isolated fourth ventricle following ventriculo - peritoneal shunting9 ) .
however , in the present case , there were no complications caused by the catheter during follow - up .
we report a case of hydrocephalus associated with neurosarcoidosis treated by multiple shunt placement with clinical features and a review of literature .
 a 31-year - old man was admitted to our hospital due to hydrocephalus with neurosarcoidosis . 
 ventriculo - peritoneal shunting was performed in the right lateral ventricle with intravenous methylprednisolone . 
 subsequently , after 4 months , additional ventriculo - peritoneal shunting in the left lateral ventricle was performed due to the enlarged left lateral ventricle and slit - like right lateral ventricle . 
 after 6 months , he was re - admitted due to upward gaze palsy , and magnetic resonance image showed an isolated fourth ventricle with both the inlet and outlet of fourth ventricle obstructed by recurrent neurosarcoidosis . owing to the difficulty of using an endoscope , we performed neuronavigator - guided ventriculo - peritoneal shunting via the left lateral transcerebellar approach for the treatment of the isolated fourth ventricle with intravenous methyl prednisolone . 
 the patient was discharged with improved neurological status . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION