Acute Cerebellitis as a Rare Treatable Cause of Obstructive Hydrocephalus.

Acute cerebellitis commonly presents as acute ataxia in children. Rarely, it can result in obstructive hydrocephalus. We report a 10-year-old boy with Mycoplasma infection-associated acute cerebellitis and obstructive hydrocephalus that responded well to conservative management with no residual neurological deficit. Wide recognition of this treatable clinical entity among neurologist would avert unnecessary investigations and ensure rationale management. Copyright:

Introduction

Acute cerebellitis is a rare inflammatory syndrome presenting in children with acute ataxia. It may occur due to some primary infection or as a postinfectious or postvaccination disorder. Various infectious agents implicated in acute cerebellitis include varicella-zoster virus, Epstein–Barr virus (EBV), mumps virus, rubella virus, Coxsackie virus, Coxiella burnetti, and Mycoplasma pneumonia.[123]M. pneumonia–related acute cerebellitis can seldom result in obstructive hydrocephalus and tonsillar herniation requiring surgical intervention like external ventricular drainage.[45678] We report a similar case of acute cerebellitis and obstructive hydrocephalus managed conservatively without any surgical intervention.

Case Report

A 10-year-old boy was admitted with complaints of severe headache, vomiting, and irritability for 4 days. Headache was diffuse, severe in intensity, throbbing in nature, persisted throughout the day, interfered with his sleep and daily activity, and could not be relieved with oral analgesics. Headache was also associated with multiple episodes of projectile vomiting and irritability. There was no history of recent vaccination or head trauma. There was no history of seizures, loss of consciousness, although he remained irritable. Around 2 weeks before the onset of these symptoms, he had fever, cough, and cold that lasted for 3–4 days.

On examination, the child was afebrile (98.2°F) with physiological pulse rate (86/min), respiratory rate (30/min), and blood pressure (100/62 mm Hg). His anthropometric parameters were within the normal range. There was no pallor, icterus, or any other significant finding on general physical examination. At admission, he was conscious, irritable but comprehended verbal commands. There was no evidence of cranial nerve palsies, and fundoscopy revealed marginal blurring of disc margins. Motor system examination did not show any focal neurological deficit. Around 2 weeks before the onset of headache, he had fever, cough, and cold that lasted for 3–4 days. There were no signs of meningeal irritation. Given early signs of encephalopathy and features of raised intracranial pressure, measures including mannitol, dexamethasone, and broad-spectrum antibiotics including ceftriaxone and vancomycin were started.

Preliminary laboratory tests showed mild leukocytosis with neutrophilia (total white cell count 13,000/mm3; neutrophils 83%), normal serum sodium (137 mEq/L), serum alanine aminotransferase levels (36 IU/L), serum bilirubin (0.9mg/dL), and serum creatinine (0.7mg/dL) levels. Magnetic resonance imaging (MRI) of his brain revealed mild obstructive hydrocephalus with periventricular ooze, ill-defined altered signal intensity area with contrast enhancement in the bilateral cerebellum, and mild tonsillar herniation (Figure 1).

Figure 1

MRI brain T2 fluid-attenuated inversion recovery image reveals hyperintensity in the bilateral cerebellar hemisphere (A) with prominent temporal horns, and dilated third and lateral ventricle with periventricular ooze (B). Repeat MRI brain performed 3 months later revealed minimal hyperintense cerebellar signals (C) with complete resolution of hydrocephalus (D)

Once the clinical signs of raised intracranial pressure improved, a guarded lumbar puncture was performed. Cerebrospinal fluid examination showed pleocytosis (60–70 white blood cells/mm3, predominantly lymphocytes), proteins 31mg/dL, glucose 84mg/dL (94mg/dL blood sugars), and culture being sterile. The blood culture was sterile. Considering the postinfectious cause for acute cerebellitis, serological tests for M. pneumonia and EBV were also performed. Serum was tested for Immunoglobulin M (IgM) antibodies against M. pneumoniae using enzyme immunoassay and the test result was found to be positive. Serologic tests for EBV were negative. Other viral panels could not be performed owing to financial constraints. Antibiotics were revised to azithromycin (10mg/kg/day), which continued for the next 10 days. His vomiting had subsided, and headache had improved at the time of discharge. At 3-month follow-up, repeat imaging revealed the resolution of hydrocephalus.

Discussion

Acute cerebellitis is a neurological condition characterized by acute onset of cerebellar dysfunction along with fever, nausea, headache, and altered mental status, with MRI showing evidence of cerebellar inflammation.[9] Among the various etiological agents, Mycoplasma is an uncommon cause of cerebellitis. Majority of the reported cases had required surgical intervention. The index case resolved completely with conservative management.

The organism has been considered to result in neurological manifestation owing to postinfectious, immune-mediated central nervous system (CNS) inflammation rather than dissemination of the organism to the brain. As the infective organism does not actively propagate at the site of inflammation in neurologic diseases, the therapeutic role of macrolides in the treatment of neurologic disorders due to M. pneumoniae infection is unclear. However, macrolides may indirectly contribute to clinical improvement by eliminating the additional supply of the harmful bacterial components, causing Inflammation. Antimicrobial treatment, especially macrolides, is found to be sufficient for CNS involvement associated with M. pneumonia along with the steroids. This patient also showed rapid recovery in the first week of treatment with intravenous steroids, osmotic diuretic (mannitol), and azithromycin. So it is difficult to say whether steroids or azithromycin worked in this case.

Akin to the index case, in a series of patients with acute cerebellitis and obstructive hydrocephalus associated with M. pneumonia infection, IgM antibodies were positive in all five cases.[1234] Only Schmuker et al.[4] reported Mycoplasma DNA in throat swab in addition to positive serology. Neurological and cognitive sequelae are common in children with acute cerebellitis. They range from ataxia to mild tremors and cognitive decline in spatial visualization ability, language skills, and concentration.[1234] Fortunately, the index case at 3 months had no cognitive, behavioral, or neurological deficits.

In conclusion, M. pneumoniae cerebellitis though rare may be associated with acute hydrocephalus and tonsillar herniation. This report adds to the benign form of Mycoplasma-associated acute cerebellitis that resolved with conservative management with reversal of obstructive hydrocephalus and no neurological deficit. Wide recognition of this treatable clinical entity among neurologist would avert unnecessary investigations and ensure rationale management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.